Kv7.1
2168 literature references associated to Kv7.1
1
Lang CN
et al.
Transgenic rabbit models to investigate the cardiac ion channel disease long QT syndrome.
Prog. Biophys. Mol. Biol.,
2016
May
19
, ().
2
Lan X
et al.
Grafting voltage and pharmacological sensitivity in potassium channels.
Cell Res.,
2016
May
13
, ().
3
Ali A
et al.
Heat shock protein 70 gene polymorphisms' influence on the electrophysiology of long QT syndrome.
J Interv Card Electrophysiol,
2016
Mar
, 45 (119-30).
4
Jiang M
et al.
JPH-2 interacts with Cai-handling proteins and ion channels in dyads: Contribution to premature ventricular contraction-induced cardiomyopathy.
Heart Rhythm,
2016
Mar
, 13 (743-752).
5
Carbonell-Pascual B
et al.
Comparison between Hodgkin-Huxley and Markov formulations of cardiac ion channels.
J. Theor. Biol.,
2016
Jun
21
, 399 (92-102).
6
Yamaguchi Y
et al.
Latent pathogenicity of the G38S polymorphism of KCNE1 K(+) channel modulator.
Heart Vessels,
2016
Jun
2
, ().
7
Izumi G
et al.
Compound Mutations Cause Increased Cardiac Events in Children with Long QT Syndrome: Can the Sequence Homology-Based Tools be Applied for Prediction of Phenotypic Severity?
Pediatr Cardiol,
2016
Jun
, 37 (962-70).
8
Wu W
et al.
Molecular Basis of Cardiac Delayed Rectifier Potassium Channel Function and Pharmacology.
Card Electrophysiol Clin,
2016
Jun
, 8 (275-84).
9
Major P
et al.
A novel transgenic rabbit model with reduced repolarization reserve: long QT syndrome caused by a dominant-negative mutation of the KCNE1 gene.
Br. J. Pharmacol.,
2016
Jun
, 173 (2046-61).
10
Roy PM
et al.
Anaesthesia management of a case of Jervell and Lange-Nielsen syndrome for minimally invasive bilateral thoracoscopic cervicothoracic sympathectomy.
Indian J Anaesth,
2016
Jun
, 60 (424-6).
11
Shao H
et al.
[Relationship between electrocardiographic and genetic mutation (MYH7-H1717Q, MYLK2-K324E and KCNQ1-R190W) phenotype in patients with hypertrophic cardiomyopathy].
Zhonghua Xin Xue Guan Bing Za Zhi,
2016
Jan
24
, 44 (50-4).
12
Bodi I
et al.
Mechanisms of acquired long-QT syndrome in patients with propionic acidemia.
Heart Rhythm,
2016
Feb
5
, ().
13
Al-Hazza A
et al.
Upregulation of basolateral small conductance potassium channels (KCNQ1/KCNE3) in ulcerative colitis.
Biochem. Biophys. Res. Commun.,
2016
Feb
5
, 470 (473-8).
14
Neubauer J
et al.
Post-mortem whole-exome sequencing (WES) with a focus on cardiac disease-associated genes in five young sudden unexplained death (SUD) cases.
Int. J. Legal Med.,
2016
Feb
4
, ().
15
Fang P
et al.
[Progress in research on defective protein trafficking and functional restoration in HERG-associated long QT syndrome].
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2016
Feb
, 33 (101-4).
16
Rannals MD
et al.
Psychiatric Risk Gene Transcription Factor 4 Regulates Intrinsic Excitability of Prefrontal Neurons via Repression of SCN10a and KCNQ1.
Neuron,
2016
Apr
6
, 90 (43-55).
17
Crumb WJ
et al.
An evaluation of 30 clinical drugs against the comprehensive in vitro proarrhythmia assay (CiPA) proposed ion channel panel.
J Pharmacol Toxicol Methods,
2016
Apr
6
, ().
18
Choi JI
et al.
α1-Syntrophin Variant Identified in Drug-Induced Long QT Syndrome Increases Late Sodium Current.
PLoS ONE,
2016
, 11 (e0152355).
19
Cubeddu LX
Drug-induced Inhibition and Trafficking Disruption of ion Channels: Pathogenesis of QT Abnormalities and Drug-induced Fatal Arrhythmias.
Curr Cardiol Rev,
2016
, 12 (141-54).
20
Murray CI
et al.
Unnatural amino acid photo-crosslinking of the IKs channel complex demonstrates a KCNE1:KCNQ1 stoichiometry of up to 4:4.
Elife,
2016
, 5 ().
21
Ichikawa M
et al.
Multigenerational Inheritance of Long QT Syndrome Type 2 in a Japanese Family.
Intern. Med.,
2016
, 55 (259-62).
22
Zhao Y
et al.
Regulation of SCN3B/scn3b by Interleukin 2 (IL-2): IL-2 modulates SCN3B/scn3b transcript expression and increases sodium current in myocardial cells.
BMC Cardiovasc Disord,
2016
, 16 (1).
23
Ware WA
et al.
Sudden death associated with QT interval prolongation and KCNQ1 gene mutation in a family of English Springer Spaniels.
J. Vet. Intern. Med.,
2015 Mar-Apr
, 29 (561-8).
24
Neverisky DL
et al.
Ion channel-transporter interactions.
Crit. Rev. Biochem. Mol. Biol.,
2015 Jul-Aug
, 51 (257-67).
25
Neethling A
et al.
Filamin C: a novel component of the KCNE2 interactome during hypoxia.
Cardiovasc J Afr,
2015 Jan-Feb
, 27 (4-11).
26
Hertz CL
et al.
Genetic investigations of sudden unexpected deaths in infancy using next-generation sequencing of 100 genes associated with cardiac diseases.
Eur. J. Hum. Genet.,
2015
Sep
9
, ().
27
Coll M
et al.
Genetic investigation of sudden unexpected death in epilepsy cohort by panel target resequencing.
Int. J. Legal Med.,
2015
Sep
30
, ().
28
Abbott GW
KCNE1 and KCNE3: The yin and yang of voltage-gated K(+) channel regulation.
Gene,
2015
Sep
26
, ().
29
El-Sherif N
et al.
Role of pharmacotherapy in cardiac ion channelopathies.
Pharmacol. Ther.,
2015
Sep
12
, ().
30
Provence A
et al.
The Novel KV7.2/KV7.3 Channel Opener ICA-069673 Reveals Subtype-Specific Functional Roles in Guinea Pig Detrusor Smooth Muscle Excitability and Contractility.
J. Pharmacol. Exp. Ther.,
2015
Sep
, 354 (290-301).
31
Hu Y
et al.
Engineering a peptide inhibitor towards the KCNQ1/KCNE1 potassium channel (IKs).
Peptides,
2015
Sep
, 71 (77-83).
32
Sahu ID
et al.
Probing Structural Dynamics and Topology of the KCNE1 Membrane Protein in Lipid Bilayers via Site-Directed Spin Labeling and Electron Paramagnetic Resonance Spectroscopy.
Biochemistry,
2015
Oct
20
, 54 (6402-12).
33
Frea S
et al.
New echocardiographic insights in short QT syndrome: More than a channelopathy?
Heart Rhythm,
2015
Oct
, 12 (2096-105).
34
Andersen MN
et al.
Protein kinase A stimulates Kv7.1 surface expression by regulating Nedd4-2-dependent endocytic trafficking.
Am. J. Physiol., Cell Physiol.,
2015
Nov
15
, 309 (C693-706).
35
Kang Y
et al.
Regulation of the human ether-a-go-go-related gene (hERG) potassium channel by Nedd4 family interacting proteins (Ndfips).
Biochem. J.,
2015
Nov
15
, 472 (71-82).
36
Bakker B
et al.
A Girl With Beckwith-Wiedemann Syndrome and Pseudohypoparathyroidism Type 1B Due to Multiple Imprinting Defects.
J. Clin. Endocrinol. Metab.,
2015
Nov
, 100 (3963-6).
37
Zoledziewska M
et al.
Height-reducing variants and selection for short stature in Sardinia.
Nat. Genet.,
2015
Nov
, 47 (1352-6).
38
Powell KL
et al.
In response: LQT, HCN, and epilepsy model.
Epilepsia,
2015
Nov
, 56 (1855-6).
40
Vassilakopoulou V
et al.
Distinctive malfunctions of calmodulin mutations associated with heart RyR2-mediated arrhythmic disease.
Biochim. Biophys. Acta,
2015
Nov
, 1850 (2168-76).
41
Liin SI
et al.
Polyunsaturated fatty acid analogs act antiarrhythmically on the cardiac IKs channel.
Proc. Natl. Acad. Sci. U.S.A.,
2015
May
5
, 112 (5714-9).
42
Qian Y
et al.
Joint effect of CENTD2 and KCNQ1 polymorphisms on the risk of type 2 diabetes mellitus among Chinese Han population.
Mol. Cell. Endocrinol.,
2015
May
15
, 407 (46-51).
43
Matus M
et al.
Upregulation of SERCA2a following short-term ACE inhibition (by enalaprilat) alters contractile performance and arrhythmogenicity of healthy myocardium in rat.
Mol. Cell. Biochem.,
2015
May
, 403 (199-208).
44
Leren IS
et al.
Cardiac Mechanical Alterations and Genotype Specific Differences in Subjects With Long QT Syndrome.
JACC Cardiovasc Imaging,
2015
May
, 8 (501-10).
45
Jahangir A
et al.
Strain Echocardiography and LQTS Subtypes: Mechanical Alterations in an Electrical Disorder.
JACC Cardiovasc Imaging,
2015
May
, 8 (511-3).
46
Kolder IC
et al.
Analysis for Genetic Modifiers of Disease Severity in Patients with Long QT Syndrome Type 2.
Circ Cardiovasc Genet,
2015
Mar
3
, ().
47
Perry MD
et al.
Getting to the heart of hERG K(+) channel gating.
J. Physiol. (Lond.),
2015
Mar
27
, ().
48
Goodchild SJ
et al.
Sequence of gating charge movement and pore gating in HERG activation and deactivation pathways.
Biophys. J.,
2015
Mar
24
, 108 (1435-47).
49
Biliczki P
et al.
The interaction between delayed rectifier channel alpha-subunits does not involve hetero-tetramer formation.
Naunyn Schmiedebergs Arch. Pharmacol.,
2015
Mar
20
, ().
50
Steffensen AB
et al.
IKs Gain- and Loss-of-Function In Early-Onset Lone Atrial Fibrillation.
J. Cardiovasc. Electrophysiol.,
2015
Mar
19
, ().
51
Villoutreix BO
et al.
Computational investigations of hERG channel blockers: New insights and current predictive models.
Adv. Drug Deliv. Rev.,
2015
Mar
12
, ().
52
Laurentino S
et al.
Epigenetic germline mosaicism in infertile men.
Hum. Mol. Genet.,
2015
Mar
1
, 24 (1295-304).
53
Gómez-Úriz AM
et al.
Obesity and ischemic stroke modulate the methylation levels of KCNQ1 in white blood cells.
Hum. Mol. Genet.,
2015
Mar
1
, 24 (1432-40).
54
Wang W
et al.
Identification of a key residue in Kv7.1 potassium channel essential for sensing external potassium ions.
J. Gen. Physiol.,
2015
Mar
, 145 (201-12).
55
Hiippala A
et al.
Expanding the phenotype of Timothy syndrome type 2: an adolescent with ventricular fibrillation but normal development.
Am. J. Med. Genet. A,
2015
Mar
, 167A (629-34).
56
Huang J
et al.
Genetic variants in KCNE1, KCNQ1, and NOS1AP in sudden unexplained death during daily activities in Chinese Han population.
J. Forensic Sci.,
2015
Mar
, 60 (351-6).
57
Wemhöner K
et al.
Gain-of-function mutations in the calcium channel CACNA1C (Cav1.2) cause non-syndromic long-QT but not Timothy syndrome.
J. Mol. Cell. Cardiol.,
2015
Mar
, 80 (186-95).
58
Stott JB
et al.
Contribution of kv7 channels to natriuretic Peptide mediated vasodilation in normal and hypertensive rats.
Hypertension,
2015
Mar
, 65 (676-82).
59
Yang L
et al.
Intravenous anesthetic propofol inhibits multiple human cardiac potassium channels.
Anesthesiology,
2015
Mar
, 122 (571-84).
60
Hayashi K
et al.
Functional Characterization of Rare Variants Implicated in Susceptibility to Lone Atrial Fibrillation.
Circ Arrhythm Electrophysiol,
2015
Jun
30
, ().
61
Liin SI
et al.
The KCNQ1 channel - remarkable flexibility in gating allows for functional versatility.
J. Physiol. (Lond.),
2015
Jun
15
, 593 (2605-15).
62
Fu Y
et al.
Association between maternal single nucleotide polymorphisms in genes regulating glucose metabolism and risk for neural tube defects in offspring.
Birth Defects Res. Part A Clin. Mol. Teratol.,
2015
Jun
, 103 (471-8).
63
Mills TA
et al.
Activation of KV7 channels stimulates vasodilatation of human placental chorionic plate arteries.
Placenta,
2015
Jun
, 36 (638-44).
64
Kouvaros S
et al.
Hippocampal sharp waves and ripples: Effects of aging and modulation by NMDA receptors and L-type Ca2+ channels.
Neuroscience,
2015
Jul
9
, 298 (26-41).
65
Asahara S
et al.
Paternal allelic mutation at the Kcnq1 locus reduces pancreatic β-cell mass by epigenetic modification of Cdkn1c.
Proc. Natl. Acad. Sci. U.S.A.,
2015
Jul
7
, 112 (8332-7).
66
Stattin EL
et al.
Genetic screening in sudden cardiac death in the young can save future lives.
Int. J. Legal Med.,
2015
Jul
31
, ().
67
Harrell DT
et al.
Genotype-dependent differences in age of manifestation and arrhythmia complications in short QT syndrome.
Int. J. Cardiol.,
2015
Jul
1
, 190 (393-402).
68
Wu ZJ
et al.
Characterization of a Chinese KCNQ1 mutation (R259H) that shortens repolarization and causes short QT syndrome 2.
J Geriatr Cardiol,
2015
Jul
, 12 (394-401).
69
Kienitz MC
et al.
Synergistic modulation of KCNQ1/KCNE1 K(+) channels (IKs) by phosphatidylinositol 4,5-bisphosphate (PIP2) and [ATP]i.
Cell. Signal.,
2015
Jul
, 27 (1457-68).
70
Issa NP
et al.
QT interval prolongation in a patient with LQT2 on levetiracetam.
Seizure,
2015
Jul
, 29 (134-6).
71
72
Hiramoto M
et al.
Comparative analysis of type 2 diabetes-associated SNP alleles identifies allele-specific DNA-binding proteins for the KCNQ1 locus.
Int. J. Mol. Med.,
2015
Jul
, 36 (222-30).
73
Yoshizawa S
et al.
Pyrrole-imidazole polyamide-mediated silencing of KCNQ1OT1 expression induces cell death in Wilms' tumor cells.
Int. J. Oncol.,
2015
Jul
, 47 (115-21).
74
Li L
et al.
Genetic variants of potassium voltage-gated channel genes (KCNQ1, KCNH2, and KCNE1) affected the risk of atrial fibrillation in elderly patients.
Genet Test Mol Biomarkers,
2015
Jul
, 19 (359-65).
75
Vucic E
et al.
Kir1.1 (ROMK) and Kv7.1 (KCNQ1/KvLQT1) are essential for normal gastric acid secretion: importance of functional Kir1.1.
Pflugers Arch.,
2015
Jul
, 467 (1457-68).
76
Xu Y
et al.
Probing binding sites and mechanisms of action of an I(Ks) activator by computations and experiments.
Biophys. J.,
2015
Jan
6
, 108 (62-75).
77
Zhang J
et al.
Electrophysiological and trafficking defects of the SCN5A T353I mutation in Brugada syndrome are rescued by alpha-allocryptopine.
Eur. J. Pharmacol.,
2015
Jan
5
, 746 (333-43).
78
Chong E
et al.
Resveratrol, a red wine antioxidant, reduces atrial fibrillation susceptibility in the failing heart by PI3K/AKT/eNOS signaling pathway activation.
Heart Rhythm,
2015
Jan
30
, ().
79
Nakajo K
et al.
KCNQ1 channel modulation by KCNE proteins via the voltage-sensing domain.
J. Physiol. (Lond.),
2015
Jan
21
, ().
80
Gayen S
et al.
Structural analysis of the S4-S5 linker of the human KCNQ1 potassium channel.
Biochem. Biophys. Res. Commun.,
2015
Jan
2
, 456 (410-4).
81
ter Bekke RM
et al.
Electromechanical window negativity in genotyped long-QT syndrome patients: relation to arrhythmia risk.
Eur. Heart J.,
2015
Jan
14
, 36 (179-86).
82
Sedivy V
et al.
Role of Kv7 channels in responses of the pulmonary circulation to hypoxia.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2015
Jan
1
, 308 (L48-57).
83
Costello JP
et al.
Surgical cardiac denervation therapy for treatment of congenital ion channelopathies in pediatric patients: a contemporary, single institutional experience.
World J Pediatr Congenit Heart Surg,
2015
Jan
, 6 (33-8).
84
Xiong Q
et al.
Arrhythmogenic cardiomyopathy in a patient with a rare loss-of-function KCNQ1 mutation.
J Am Heart Assoc,
2015
Jan
, 4 (e001526).
86
Riuró H
et al.
Genetic analysis, in silico prediction, and family segregation in long QT syndrome.
Eur. J. Hum. Genet.,
2015
Jan
, 23 (79-85).
87
Kim TY
et al.
Complex excitation dynamics underlie polymorphic ventricular tachycardia in a transgenic rabbit model of long QT syndrome type 1.
Heart Rhythm,
2015
Jan
, 12 (220-8).
88
Schultz BM
et al.
Enhancers compete with a long non-coding RNA for regulation of the Kcnq1 domain.
Nucleic Acids Res.,
2015
Jan
, 43 (745-59).
89
Porta A
et al.
Autonomic control of heart rate and QT interval variability influences arrhythmic risk in long QT syndrome type 1.
J. Am. Coll. Cardiol.,
2015
Feb
3
, 65 (367-74).
90
Moreno C
et al.
Marine n-3 PUFAs modulate IKs gating, channel expression, and location in membrane microdomains.
Cardiovasc. Res.,
2015
Feb
1
, 105 (223-32).
91
O-Uchi J
et al.
Impaired IKs channel activation by Ca(2+)-dependent PKC shows correlation with emotion/arousal-triggered events in LQT1.
J. Mol. Cell. Cardiol.,
2015
Feb
, 79 (203-11).
92
Tseng GN
et al.
Understanding the microscopic mechanisms for LQT1 needs a global view of the I(Ks) channel.
Heart Rhythm,
2015
Feb
, 12 (395-6).
93
Eldstrom J
et al.
Microscopic mechanisms for long QT syndrome type 1 revealed by single-channel analysis of I(Ks) with S3 domain mutations in KCNQ1.
Heart Rhythm,
2015
Feb
, 12 (386-94).
94
de Llano CT
et al.
Further evidence of the association between LQT syndrome and epilepsy in a family with KCNQ1 pathogenic variant.
Seizure,
2015
Feb
, 25 (65-7).
95
Lee S
et al.
Heterogeneity in Kv7 channel function in the cerebral and coronary circulation.
Microcirculation,
2015
Feb
, 22 (109-21).
96
Winbo A
et al.
Vestibular dysfunction is a clinical feature of the Jervell and Lange-Nielsen Syndrome.
Scand. Cardiovasc. J.,
2015
Feb
, 49 (7-13).
97
Anderson HN
et al.
Marked, transient, emotion-triggered QT accentuation in an adolescent female with type 1 long QT syndrome.
Cardiol Young,
2015
Feb
, 25 (376-9).
98
Nekouzadeh A
et al.
Conformational changes of an ion-channel during gating and emerging electrophysiologic properties: Application of a computational approach to cardiac Kv7.1.
Prog. Biophys. Mol. Biol.,
2015
Dec
30
, ().
99
Zhu W
et al.
Molecular motions that shape the cardiac action potential: Insights from voltage clamp fluorometry.
Prog. Biophys. Mol. Biol.,
2015
Dec
25
, ().
100
Peters CH
et al.
Triggers for arrhythmogenesis in the Brugada and long QT 3 syndromes.
Prog. Biophys. Mol. Biol.,
2015
Dec
20
, ().
101
Slaats GG
et al.
Screen-based identification and validation of four new ion channels as regulators of renal ciliogenesis.
J. Cell. Sci.,
2015
Dec
15
, 128 (4550-9).
102
Robyns T
et al.
Targeted capture sequencing in a large LQTS family reveals a new pathogenic mutation c.2038delG in KCNH2 initially missed due to allelic dropout.
Acta Cardiol,
2015
Dec
, 70 (747-9).
103
Qureshi SF
et al.
Mutational analysis of SCN5A gene in long QT syndrome.
Meta Gene,
2015
Dec
, 6 (26-35).
104
Kanters JK
et al.
Combined gating and trafficking defect in Kv11.1 manifests as a malignant long QT syndrome phenotype in a large Danish p.F29L founder family.
Scand. J. Clin. Lab. Invest.,
2015
Dec
, 75 (699-709).
105
Wasano K
et al.
A novel frameshift mutation in KCNQ4 in a family with autosomal recessive non-syndromic hearing loss.
Biochem. Biophys. Res. Commun.,
2015
Aug
7
, 463 (582-6).
106
Zhi D
et al.
The enhancement of cardiac toxicity by concomitant administration of Berberine and macrolides.
Eur J Pharm Sci,
2015
Aug
30
, 76 (149-55).
107
Yuan J
et al.
Potassium channel KCNJ15 is required for histamine-stimulated gastric acid secretion.
Am. J. Physiol., Cell Physiol.,
2015
Aug
15
, 309 (C264-70).
108
Anneken L
et al.
Estradiol regulates human QT-interval: acceleration of cardiac repolarization by enhanced KCNH2 membrane trafficking.
Eur. Heart J.,
2015
Aug
12
, ().
109
Koponen M
et al.
Follow-up of 316 molecularly defined pediatric long-QT syndrome patients: clinical course, treatments, and side effects.
Circ Arrhythm Electrophysiol,
2015
Aug
, 8 (815-23).
110
Krönauer T
et al.
[Perioperative treatment of patients with long QT syndrome].
Anaesthesist,
2015
Aug
, 64 (625-39).
111
Imam MH
et al.
A novel technique to investigate the effect of ageing on ventricular repolarization characteristics in healthy and LQTS subjects.
Conf Proc IEEE Eng Med Biol Soc,
2015
Aug
, 2015 (2796-9).
112
Li G
et al.
RNA interference-based therapeutics for inherited long QT syndrome.
Exp Ther Med,
2015
Aug
, 10 (395-400).
113
Krönauer T
et al.
[Long QT syndrome : History, genetics, clinical symptoms, causes and therapy].
Anaesthesist,
2015
Aug
, 64 (586-95).
114
Winbo A
et al.
Third trimester fetal heart rate predicts phenotype and mutation burden in the type 1 long QT syndrome.
Circ Arrhythm Electrophysiol,
2015
Aug
, 8 (806-14).
115
Potet F
et al.
Intracellular calcium attenuates late current conducted by mutant human cardiac sodium channels.
Circ Arrhythm Electrophysiol,
2015
Aug
, 8 (933-41).
116
Chang Q
et al.
Virally mediated Kcnq1 gene replacement therapy in the immature scala media restores hearing in a mouse model of human Jervell and Lange-Nielsen deafness syndrome.
EMBO Mol Med,
2015
Aug
, 7 (1077-86).
117
Lampert R
Reassuring News for Genetically Tested, Appropriately Treated, Low-Risk LQTS Patients.
J. Cardiovasc. Electrophysiol.,
2015
Aug
, 26 (859-61).
118
Chen J
et al.
SjAPI-2 is the first member of a new neurotoxin family with Ascaris-type fold and KCNQ1 inhibitory activity.
Int. J. Biol. Macromol.,
2015
Aug
, 79 (504-10).
119
Osterbur ML
et al.
An Interdomain KCNH2 Mutation Produces an Intermediate Long QT Syndrome.
Hum. Mutat.,
2015
Aug
, 36 (764-73).
120
Zhang C
et al.
Identification of Low-Risk Adult Congenital LQTS Patients.
J. Cardiovasc. Electrophysiol.,
2015
Aug
, 26 (853-8).
121
Christ T
et al.
LQT1-phenotypes in hiPSC: Are we measuring the right thing?
Proc. Natl. Acad. Sci. U.S.A.,
2015
Apr
21
, 112 (E1968).
122
Greber B
et al.
Reply to Christ et al.: LQT1 and JLNS phenotypes in hiPSC-derived cardiomyocytes are due to KCNQ1 mutations.
Proc. Natl. Acad. Sci. U.S.A.,
2015
Apr
21
, 112 (E1969).
123
Morales-Cano D
et al.
Kv7 channels critically determine coronary artery reactivity: left-right differences and down-regulation by hyperglycaemia.
Cardiovasc. Res.,
2015
Apr
1
, 106 (98-108).
124
Kapplinger JD
et al.
Enhancing the Predictive Power of Mutations in the C-Terminus of the KCNQ1-Encoded Kv7.1 Voltage-Gated Potassium Channel.
J Cardiovasc Transl Res,
2015
Apr
, 8 (187-97).
125
Williams VS
et al.
Multiplex ligation-dependent probe amplification copy number variant analysis in patients with acquired long QT syndrome.
Europace,
2015
Apr
, 17 (635-41).
126
Lau E
et al.
Spatially Discordant Alternans and Arrhythmias in Tachypacing-Induced Cardiac Myopathy in Transgenic LQT1 Rabbits: The Importance of IKs and Ca2+ Cycling.
PLoS ONE,
2015
, 10 (e0122754).
127
Kasimova MA
et al.
PIP₂-dependent coupling is prominent in Kv7.1 due to weakened interactions between S4-S5 and S6.
Sci Rep,
2015
, 5 (7474).
128
Kong X
et al.
The Association of Type 2 Diabetes Loci Identified in Genome-Wide Association Studies with Metabolic Syndrome and Its Components in a Chinese Population with Type 2 Diabetes.
PLoS ONE,
2015
, 10 (e0143607).
129
Pedersen PJ
et al.
Molecular Cloning and Functional Expression of the Equine K+ Channel KV11.1 (Ether à Go-Go-Related/KCNH2 Gene) and the Regulatory Subunit KCNE2 from Equine Myocardium.
PLoS ONE,
2015
, 10 (e0138320).
130
Ao D
et al.
The rs2237892 Polymorphism in KCNQ1 Influences Gestational Diabetes Mellitus and Glucose Levels: A Case-Control Study and Meta-Analysis.
PLoS ONE,
2015
, 10 (e0128901).
131
Mayo S
et al.
In Pursuit of New Imprinting Syndromes by Epimutation Screening in Idiopathic Neurodevelopmental Disorder Patients.
Biomed Res Int,
2015
, 2015 (341986).
132
Liu X
et al.
Effects of IGF2BP2, KCNQ1 and GCKR polymorphisms on clinical outcome in metastatic gastric cancer treated with EOF regimen.
Pharmacogenomics,
2015
, 16 (959-70).
133
Itoh H
et al.
A Common Mutation of Long QT Syndrome Type 1 in Japan.
Circ. J.,
2015
, 79 (2026-30).
134
Inanobe A
et al.
An Epithelial Ca2+-Sensor Protein is an Alternative to Calmodulin to Compose Functional KCNQ1 Channels.
Cell. Physiol. Biochem.,
2015
, 36 (1847-61).
135
Allegue C
et al.
Genetic Analysis of Arrhythmogenic Diseases in the Era of NGS: The Complexity of Clinical Decision-Making in Brugada Syndrome.
PLoS ONE,
2015
, 10 (e0133037).
136
Abete I
et al.
Epigenetic Changes in the Methylation Patterns of KCNQ1 and WT1 after a Weight Loss Intervention Program in Obese Stroke Patients.
Curr Neurovasc Res,
2015
, 12 (321-33).
137
Koppes E
et al.
Partial Loss of Genomic Imprinting Reveals Important Roles for Kcnq1 and Peg10 Imprinted Domains in Placental Development.
PLoS ONE,
2015
, 10 (e0135202).
138
Warsi J
et al.
Regulation of Voltage Gated K+ Channel KCNE1/KCNQ1 by the Janus Kinase JAK3.
Cell. Physiol. Biochem.,
2015
, 37 (2476-85).
139
Cazals Y
et al.
KCNK5 channels mostly expressed in cochlear outer sulcus cells are indispensable for hearing.
Nat Commun,
2015
, 6 (8780).
140
Bellin M
et al.
Human iPS cell models of Jervell and Lange-Nielsen syndrome.
Rare Dis,
2015
, 3 (e1012978).
141
Huerta-Chagoya A
et al.
Genetic determinants for gestational diabetes mellitus and related metabolic traits in Mexican women.
PLoS ONE,
2015
, 10 (e0126408).
142
Hu RM
et al.
Arrhythmogenic Biophysical Phenotype for SCN5A Mutation S1787N Depends upon Splice Variant Background and Intracellular Acidosis.
PLoS ONE,
2015
, 10 (e0124921).
143
Liu QN
et al.
Eag Domains Regulate LQT Mutant hERG Channels in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes.
PLoS ONE,
2015
, 10 (e0123951).
144
145
Svalø J
et al.
Functional and molecular evidence for Kv7 channel subtypes in human detrusor from patients with and without bladder outflow obstruction.
PLoS ONE,
2015
, 10 (e0117350).
146
Sun P
et al.
A distinct three-helix centipede toxin SSD609 inhibits I(ks) channels by interacting with the KCNE1 auxiliary subunit.
Sci Rep,
2015
, 5 (13399).
147
Chang YS
et al.
Mutation Analysis of KCNQ1, KCNH2 and SCN5A Genes in Taiwanese Long QT Syndrome Patients.
Int Heart J,
2015
, 56 (450-3).
148
Steffensen AB
et al.
High incidence of functional ion-channel abnormalities in a consecutive Long QT cohort with novel missense genetic variants of unknown significance.
Sci Rep,
2015
, 5 (10009).
149
Al-Aama JY
et al.
Genotype-phenotype analysis of Jervell and Lange-Nielsen syndrome in six families from Saudi Arabia.
Clin. Genet.,
2015
, 87 (74-9).
150
Leong IU
et al.
Assessment of the predictive accuracy of five in silico prediction tools, alone or in combination, and two metaservers to classify long QT syndrome gene mutations.
BMC Med. Genet.,
2015
, 16 (34).
151
Lazzerini PE
et al.
Long QT Syndrome: An Emerging Role for Inflammation and Immunity.
Front Cardiovasc Med,
2015
, 2 (26).
152
Kılıç E
et al.
Jervell and Lange-Nielsen syndrome with homozygous missense mutation of the KCNQ1 gene.
Turk. J. Pediatr.,
2014 Sep-Oct
, 56 (542-5).
153
Lazaraviciute G
et al.
A systematic review and meta-analysis of DNA methylation levels and imprinting disorders in children conceived by IVF/ICSI compared with children conceived spontaneously.
Hum. Reprod. Update,
2014 Nov-Dec
, 20 (840-52).
154
Mirams GR
et al.
Prediction of Thorough QT study results using action potential simulations based on ion channel screens.
J Pharmacol Toxicol Methods,
2014 Nov-Dec
, 70 (246-54).
155
Hancox JC
et al.
In silico investigation of a KCNQ1 mutation associated with familial atrial fibrillation.
J Electrocardiol,
2014 Mar-Apr
, 47 (158-65).
156
Dvir M
et al.
Long QT mutations at the interface between KCNQ1 helix C and KCNE1 disrupt I(KS) regulation by PKA and PIP₂.
J. Cell. Sci.,
2014
Sep
15
, 127 (3943-55).
157
Choi SH
et al.
Ginseng gintonin activates the human cardiac delayed rectifier K+ channel: involvement of Ca2+/calmodulin binding sites.
Mol. Cells,
2014
Sep
, 37 (656-63).
158
Imaniastuti R
et al.
Computational prediction of proarrhythmogenic effect of the V241F KCNQ1 mutation in human atrium.
Prog. Biophys. Mol. Biol.,
2014
Sep
, 116 (70-5).
159
Laksman ZW
et al.
Early repolarization is associated with symptoms in patients with type 1 and type 2 long QT syndrome.
Heart Rhythm,
2014
Sep
, 11 (1632-8).
160
Sung JY
et al.
Large deletion in KCNQ1 identified in a family with Jervell and Lange-Nielsen syndrome.
Ann Lab Med,
2014
Sep
, 34 (395-8).
161
van Hoeijen DA
et al.
Cardiac sodium channels and inherited electrophysiological disorders: an update on the pharmacotherapy.
Expert Opin Pharmacother,
2014
Sep
, 15 (1875-87).
162
Han SN
et al.
Identification and functional characterization of the human ether-a-go-go-related gene Q738X mutant associated with hereditary long QT syndrome type 2.
Int. J. Mol. Med.,
2014
Sep
, 34 (810-5).
163
Limpitikul WB
et al.
Calmodulin mutations associated with long QT syndrome prevent inactivation of cardiac L-type Ca(2+) currents and promote proarrhythmic behavior in ventricular myocytes.
J. Mol. Cell. Cardiol.,
2014
Sep
, 74 (115-24).
164
Khatami F
et al.
The mitochondrial DNA mutations associated with cardiac arrhythmia investigated in an LQTS family.
Iran J Basic Med Sci,
2014
Sep
, 17 (656-61).
165
Singhal R
et al.
Colchicine suppresses atrial fibrillation in failing heart.
Int. J. Cardiol.,
2014
Oct
20
, 176 (651-60).
166
Olde Nordkamp LR
et al.
Syncope in genotype-negative long QT syndrome family members.
Am. J. Cardiol.,
2014
Oct
15
, 114 (1223-8).
167
Wen W
et al.
Meta-analysis of genome-wide association studies in East Asian-ancestry populations identifies four new loci for body mass index.
Hum. Mol. Genet.,
2014
Oct
15
, 23 (5492-504).
168
Sahu ID
et al.
Structural investigation of the transmembrane domain of KCNE1 in proteoliposomes.
Biochemistry,
2014
Oct
14
, 53 (6392-401).
169
Spätjens RL
et al.
Long-QT mutation p.K557E-Kv7.1: dominant-negative suppression of IKs, but preserved cAMP-dependent up-regulation.
Cardiovasc. Res.,
2014
Oct
1
, 104 (216-25).
170
Cordeiro A
et al.
Relevance of genomic imprinting in intrauterine human growth expression of CDKN1C, H19, IGF2, KCNQ1 and PHLDA2 imprinted genes.
J. Assist. Reprod. Genet.,
2014
Oct
, 31 (1361-8).
171
Adler A
et al.
First report of a large duplication of the KCNQ1 gene in a patient with long QT syndrome.
Can J Cardiol,
2014
Oct
, 30 (1249.e5-7).
172
Terentyev D
et al.
Hyperphosphorylation of RyRs underlies triggered activity in transgenic rabbit model of LQT2 syndrome.
Circ. Res.,
2014
Nov
7
, 115 (919-28).
173
Anwar-Mohamed A
et al.
A human ether-á-go-go-related (hERG) ion channel atomistic model generated by long supercomputer molecular dynamics simulations and its use in predicting drug cardiotoxicity.
Toxicol. Lett.,
2014
Nov
4
, 230 (382-92).
174
Sachyani D
et al.
Structural basis of a Kv7.1 potassium channel gating module: studies of the intracellular c-terminal domain in complex with calmodulin.
Structure,
2014
Nov
4
, 22 (1582-94).
175
Li J
et al.
Induced KCNQ1 autoimmunity accelerates cardiac repolarization in rabbits: potential significance in arrhythmogenesis and antiarrhythmic therapy.
Heart Rhythm,
2014
Nov
, 11 (2092-100).
176
Al-Aama JY
et al.
De novo mutation in the KCNQ1 gene causal to Jervell and Lange-Nielsen syndrome.
Clin. Genet.,
2014
Nov
, 86 (492-5).
177
Hedley PL
et al.
MicroRNAs in cardiac arrhythmia: DNA sequence variation of MiR-1 and MiR-133A in long QT syndrome.
Scand. J. Clin. Lab. Invest.,
2014
May
8
, ().
178
Frolov RV
et al.
Celecoxib and ion channels: a story of unexpected discoveries.
Eur. J. Pharmacol.,
2014
May
5
, 730 (61-71).
179
Thomas PV
et al.
Localization and proteomic characterization of cholesterol-rich membrane microdomains in the inner ear.
J Proteomics,
2014
May
30
, 103 (178-93).
180
Mistry HD
et al.
Expression of voltage-dependent potassium channels in first trimester human placentae.
Placenta,
2014
May
, 35 (337-40).
181
Grunnet M
et al.
Kv7 channels as targets for anti-epileptic and psychiatric drug-development.
Eur. J. Pharmacol.,
2014
Mar
5
, 726 (133-7).
182
Abbott GW
et al.
KCNQ1, KCNE2, and Na+-coupled solute transporters form reciprocally regulating complexes that affect neuronal excitability.
Sci Signal,
2014
Mar
4
, 7 (ra22).
183
Wu J
et al.
A molecular mechanism for adrenergic-induced long QT syndrome.
J. Am. Coll. Cardiol.,
2014
Mar
4
, 63 (819-27).
184
Schwartz PJ
et al.
Sudden death by stress: how far under the nerves should we dig to find out why LQT1 patients die?
J. Am. Coll. Cardiol.,
2014
Mar
4
, 63 (828-30).
185
Hwang HS
et al.
Divergent regulation of ryanodine receptor 2 calcium release channels by arrhythmogenic human calmodulin missense mutants.
Circ. Res.,
2014
Mar
28
, 114 (1114-24).
186
Brueggemann LI
et al.
KCNQ (Kv7) potassium channel activators as bronchodilators: combination with a β2-adrenergic agonist enhances relaxation of rat airways.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2014
Mar
15
, 306 (L476-86).
187
Aflaki M
et al.
Exchange protein directly activated by cAMP mediates slow delayed-rectifier current remodeling by sustained β-adrenergic activation in guinea pig hearts.
Circ. Res.,
2014
Mar
14
, 114 (993-1003).
188
Shimizu W
[Current status and future perspective in inherited cardiac arrhythmias].
Nippon Rinsho,
2014
Mar
, 72 (553-63).
189
Chang RK
et al.
Genetic variants for long QT syndrome among infants and children from a statewide newborn hearing screening program cohort.
J. Pediatr.,
2014
Mar
, 164 (590-5.e1-3).
190
Girault A
et al.
Identification of KvLQT1 K+ channels as new regulators of non-small cell lung cancer cell proliferation and migration.
Int. J. Oncol.,
2014
Mar
, 44 (838-48).
191
Ki CS
et al.
A KCNQ1 mutation causes age-dependant bradycardia and persistent atrial fibrillation.
Pflugers Arch.,
2014
Mar
, 466 (529-40).
192
Shimizu T
et al.
Up-regulation of Kv7.1 channels in thromboxane A2-induced colonic cancer cell proliferation.
Pflugers Arch.,
2014
Mar
, 466 (541-8).
193
Bartos DC
et al.
A KCNQ1 mutation contributes to the concealed type 1 long QT phenotype by limiting the Kv7.1 channel conformational changes associated with protein kinase A phosphorylation.
Heart Rhythm,
2014
Mar
, 11 (459-68).
194
Jepps TA
et al.
Vasorelaxant effects of novel Kv7.4 channel enhancers ML213 and NS15370.
Br. J. Pharmacol.,
2014
Jun
9
, ().
195
Priest JR
et al.
Molecular Diagnosis of Long-QT syndrome at 10 Days of Life by Rapid Whole Genome Sequencing.
Heart Rhythm,
2014
Jun
25
, ().
196
Odening KE
et al.
How do Sex Hormones Modify Arrhythmogenesis in Long-QT Syndrome? - Sex Hormone Effects on Arrhythmogenic Substrate and Triggered Activity.
Heart Rhythm,
2014
Jun
19
, ().
197
Currò D
K+ channels as potential targets for the treatment of gastrointestinal motor disorders.
Eur. J. Pharmacol.,
2014
Jun
15
, 733 (97-101).
198
Mehta A
et al.
Re-trafficking of hERG reverses long QT syndrome 2 phenotype in human iPS-derived cardiomyocytes.
Cardiovasc. Res.,
2014
Jun
1
, 102 (497-506).
199
Yin G
et al.
Arrhythmogenic calmodulin mutations disrupt intracellular cardiomyocyte Ca2+ regulation by distinct mechanisms.
J Am Heart Assoc,
2014
Jun
, 3 (e000996).
200
Kinoshita K
et al.
A590T mutation in KCNQ1 C-terminal helix D decreases IKs channel trafficking and function but not Yotiao interaction.
J. Mol. Cell. Cardiol.,
2014
Jul
, 72 (273-80).
201
Glengarry JM
et al.
Long QT molecular autopsy in sudden infant death syndrome.
Arch. Dis. Child.,
2014
Jul
, 99 (635-40).
202
Romero L
et al.
In silico screening of the impact of hERG channel kinetic abnormalities on channel block and susceptibility to acquired long QT syndrome.
J. Mol. Cell. Cardiol.,
2014
Jul
, 72 (126-37).
203
Liu W
et al.
KCNE2 modulates cardiac L-type Ca(2+) channel.
J. Mol. Cell. Cardiol.,
2014
Jul
, 72 (208-18).
204
Riuró H
et al.
A missense mutation in the sodium channel β1b subunit reveals SCN1B as a susceptibility gene underlying long QT syndrome.
Heart Rhythm,
2014
Jul
, 11 (1202-9).
205
Jackson HA
et al.
LQTS in Northern BC: homozygosity for KCNQ1 V205M presents with a more severe cardiac phenotype but with minimal impact on auditory function.
Clin. Genet.,
2014
Jul
, 86 (85-90).
206
Oliveras A
et al.
Functional assembly of kv7.1/kv7.5 channels with emerging properties on vascular muscle physiology.
Arterioscler. Thromb. Vasc. Biol.,
2014
Jul
, 34 (1522-30).
207
Sakata S
et al.
Instability of KCNE1-D85N that causes long QT syndrome: stabilization by verapamil.
Pacing Clin Electrophysiol,
2014
Jul
, 37 (853-63).
208
Zhang H
et al.
Long noncoding RNA-mediated intrachromosomal interactions promote imprinting at the Kcnq1 locus.
J. Cell Biol.,
2014
Jan
6
, 204 (61-75).
209
Brueggemann LI
et al.
Differential protein kinase C-dependent modulation of Kv7.4 and Kv7.5 subunits of vascular Kv7 channels.
J. Biol. Chem.,
2014
Jan
24
, 289 (2099-111).
210
Lang F
et al.
Regulation of transport across cell membranes by the serum- and glucocorticoid-inducible kinase SGK1.
Mol. Membr. Biol.,
2014
Jan
14
, ().
211
D'Argenio V
et al.
DNA sequence capture and next-generation sequencing for the molecular diagnosis of genetic cardiomyopathies.
J Mol Diagn,
2014
Jan
, 16 (32-44).
212
Hata Y
et al.
Identification and characterization of a novel genetic mutation with prolonged QT syndrome in an unexplained postoperative death.
Int. J. Legal Med.,
2014
Jan
, 128 (105-15).
213
Mints Y
et al.
Single nucleotide polymorphisms in proximity to K-channel genes are associated with decreased longitudinal QTc variance.
Ann Noninvasive Electrocardiol,
2014
Jan
, 19 (63-9).
214
Mennerick S
et al.
Phosphatidylinositol 4,5-bisphosphate depletion fails to affect neurosteroid modulation of GABAA receptor function.
Psychopharmacology (Berl.),
2014
Feb
20
, ().
215
Li YY
et al.
KCNQ1 rs2237892 C→T gene polymorphism and type 2 diabetes mellitus in the Asian population: a meta-analysis of 15,736 patients.
J. Cell. Mol. Med.,
2014
Feb
, 18 (274-82).
216
Azzi S
et al.
Beckwith-Wiedemann and Russell-Silver Syndromes: from new molecular insights to the comprehension of imprinting regulation.
Curr Opin Endocrinol Diabetes Obes,
2014
Feb
, 21 (30-8).
217
Zhang Y
et al.
The SCN5A mutation A1180V is associated with electrocardiographic features of LQT3.
Pediatr Cardiol,
2014
Feb
, 35 (295-300).
218
Marquis-Nicholson R
et al.
Array comparative genomic hybridization identifies a heterozygous deletion of the entire KCNJ2 gene as a cause of sudden cardiac death.
Circ Cardiovasc Genet,
2014
Feb
, 7 (17-22).
219
Zhang M
et al.
Recessive cardiac phenotypes in induced pluripotent stem cell models of Jervell and Lange-Nielsen syndrome: disease mechanisms and pharmacological rescue.
Proc. Natl. Acad. Sci. U.S.A.,
2014
Dec
16
, 111 (E5383-92).
220
Schroder EA
et al.
Light phase-restricted feeding slows basal heart rate to exaggerate the type-3 long QT syndrome phenotype in mice.
Am. J. Physiol. Heart Circ. Physiol.,
2014
Dec
15
, 307 (H1777-85).
221
Xie R
et al.
Molecular mechanisms of calcium-sensing receptor-mediated calcium signaling in the modulation of epithelial ion transport and bicarbonate secretion.
J. Biol. Chem.,
2014
Dec
12
, 289 (34642-53).
222
Aromolaran AS
et al.
LQT1 mutations in KCNQ1 C-terminus assembly domain suppress IKs using different mechanisms.
Cardiovasc. Res.,
2014
Dec
1
, 104 (501-11).
223
Fukuyama M
et al.
Long QT syndrome type 8: novel CACNA1C mutations causing QT prolongation and variant phenotypes.
Europace,
2014
Dec
, 16 (1828-37).
224
Zhang KP
et al.
Translational toxicology and rescue strategies of the hERG channel dysfunction: biochemical and molecular mechanistic aspects.
Acta Pharmacol. Sin.,
2014
Dec
, 35 (1473-84).
225
Bond R
et al.
Congenital long QT syndrome: a case report of LQT2 and LQT13 in a neonate.
Europace,
2014
Dec
, 16 (1807).
226
Li P
et al.
The human ether-a-go-go-related gene activator NS1643 enhances epilepsy-associated KCNQ channels.
J. Pharmacol. Exp. Ther.,
2014
Dec
, 351 (596-604).
227
Salsbury G
et al.
Disruption of the potassium channel regulatory subunit KCNE2 causes iron-deficient anemia.
Exp. Hematol.,
2014
Dec
, 42 (1053-8.e1).
228
Wang Y
et al.
Genome editing of isogenic human induced pluripotent stem cells recapitulates long QT phenotype for drug testing.
J. Am. Coll. Cardiol.,
2014
Aug
5
, 64 (451-9).
229
Eckey K
et al.
Novel Kv7.1-phosphatidylinositol 4,5-bisphosphate interaction sites uncovered by charge neutralization scanning.
J. Biol. Chem.,
2014
Aug
15
, 289 (22749-58).
230
Harmer SC
et al.
Cellular mechanisms underlying the increased disease severity seen for patients with long QT syndrome caused by compound mutations in KCNQ1.
Biochem. J.,
2014
Aug
15
, 462 (133-42).
231
Spencer CI
et al.
Calcium transients closely reflect prolonged action potentials in iPSC models of inherited cardiac arrhythmia.
Stem Cell Reports,
2014
Aug
12
, 3 (269-81).
232
Imai M
et al.
Novel KCNQ1 splicing mutation in patients with forme fruste LQT1 aggravated by hypokalemia.
J Cardiol,
2014
Aug
, 64 (121-6).
233
Kukla P
et al.
Electrocardiogram in Andersen-Tawil syndrome. New electrocardiographic criteria for diagnosis of type-1 Andersen-Tawil syndrome.
Curr Cardiol Rev,
2014
Aug
, 10 (222-8).
234
Demars J
et al.
Genetic variants within the second intron of the KCNQ1 gene affect CTCF binding and confer a risk of Beckwith-Wiedemann syndrome upon maternal transmission.
J. Med. Genet.,
2014
Aug
, 51 (502-11).
235
Ng MC
et al.
Meta-analysis of genome-wide association studies in African Americans provides insights into the genetic architecture of type 2 diabetes.
PLoS Genet.,
2014
Aug
, 10 (e1004517).
236
Plant LD
et al.
Individual IKs channels at the surface of mammalian cells contain two KCNE1 accessory subunits.
Proc. Natl. Acad. Sci. U.S.A.,
2014
Apr
8
, 111 (E1438-46).
237
Gong Q
et al.
Position of premature termination codons determines susceptibility of hERG mutations to nonsense-mediated mRNA decay in long QT syndrome.
Gene,
2014
Apr
15
, 539 (190-7).
238
Weeke P
et al.
Exome sequencing implicates an increased burden of rare potassium channel variants in the risk of drug-induced long QT interval syndrome.
J. Am. Coll. Cardiol.,
2014
Apr
15
, 63 (1430-7).
239
Peng D
et al.
Purification and structural study of the voltage-sensor domain of the human KCNQ1 potassium ion channel.
Biochemistry,
2014
Apr
1
, 53 (2032-42).
240
Chadha PS
et al.
Contribution of kv7.4/kv7.5 heteromers to intrinsic and calcitonin gene-related peptide-induced cerebral reactivity.
Arterioscler. Thromb. Vasc. Biol.,
2014
Apr
, 34 (887-93).
241
Wang D
et al.
Cardiac channelopathy testing in 274 ethnically diverse sudden unexplained deaths.
Forensic Sci. Int.,
2014
Apr
, 237 (90-9).
242
Rizzo S
et al.
T-cell-mediated inflammatory activity in the stellate ganglia of patients with ion-channel disease and severe ventricular arrhythmias.
Circ Arrhythm Electrophysiol,
2014
Apr
, 7 (224-9).
243
Wang J
et al.
Association of KCNQ1 and KLF14 polymorphisms and risk of type 2 diabetes mellitus: A global meta-analysis.
Hum. Immunol.,
2014
Apr
, 75 (342-7).
244
Torekov SS
et al.
KCNQ1 long QT syndrome patients have hyperinsulinemia and symptomatic hypoglycemia.
Diabetes,
2014
Apr
, 63 (1315-25).
245
Dvir M
et al.
Recent molecular insights from mutated IKS channels in cardiac arrhythmia.
Curr Opin Pharmacol,
2014
Apr
, 15 (74-82).
246
Tester DJ
et al.
GENETICS OF LONG QT SYNDROME.
Methodist Debakey Cardiovasc J,
2014
1
, 10 (29-33).
247
Kanters JK
et al.
Flecainide provocation reveals concealed brugada syndrome in a long QT syndrome family with a novel L1786Q mutation in SCN5A.
Circ. J.,
2014
, 78 (1136-43).
248
Zaydman MA
et al.
Domain-domain interactions determine the gating, permeation, pharmacology, and subunit modulation of the IKs ion channel.
Elife,
2014
, 3 (e03606).
249
Iyer V
et al.
Modeling Tissue- and Mutation- Specific Electrophysiological Effects in the Long QT Syndrome: Role of the Purkinje Fiber.
PLoS ONE,
2014
, 9 (e97720).
250
Zaydman MA
et al.
PIP2 regulation of KCNQ channels: biophysical and molecular mechanisms for lipid modulation of voltage-dependent gating.
Front Physiol,
2014
, 5 (195).
251
Christiansen M
et al.
Mutations in Danish patients with long QT syndrome and the identification of a large founder family with p.F29L in KCNH2.
BMC Med. Genet.,
2014
, 15 (31).
252
Fröhler S
et al.
Exome sequencing helped the fine diagnosis of two siblings afflicted with atypical Timothy syndrome (TS2).
BMC Med. Genet.,
2014
, 15 (48).
253
Yamaguchi Y
et al.
Glycine/Serine polymorphism at position 38 influences KCNE1 subunit's modulatory actions on rapid and slow delayed rectifier K+ currents.
Circ. J.,
2014
, 78 (610-8).
254
Nielsen JB
et al.
Gain-of-function mutations in potassium channel subunit KCNE2 associated with early-onset lone atrial fibrillation.
Biomark Med,
2014
, 8 (557-70).
255
Yu CC
et al.
Apamin does not inhibit human cardiac Na+ current, L-type Ca2+ current or other major K+ currents.
PLoS ONE,
2014
, 9 (e96691).
256
Villafane J
et al.
Short QT syndrome manifesting with neonatal atrial fibrillation and bradycardia.
Cardiology,
2014
, 128 (236-40).
257
Li P
et al.
Differential modulations of KCNQ1 by auxiliary proteins KCNE1 and KCNE2.
Sci Rep,
2014
, 4 (4973).
258
Takahashi K
et al.
High prevalence of the SCN5A E1784K mutation in school children with long QT syndrome living on the Okinawa islands.
Circ. J.,
2014
, 78 (1974-9).
259
Miller D
et al.
Sodium channels, cardiac arrhythmia, and therapeutic strategy.
Adv. Pharmacol.,
2014
, 70 (367-92).
260
Murphy R
et al.
No evidence for copy number and methylation variation in H19 and KCNQ10T1 imprinting control regions in children born small for gestational age.
BMC Med. Genet.,
2014
, 15 (67).
261
Jimmy JJ
et al.
Clinical characteristics of patients with congenital long QT syndrome and bigenic mutations.
Chin. Med. J.,
2014
, 127 (1482-6).
263
Hennessey JA
et al.
A CACNA1C variant associated with reduced voltage-dependent inactivation, increased CaV1.2 channel window current, and arrhythmogenesis.
PLoS ONE,
2014
, 9 (e106982).
264
Shimizu W
Clinical and genetic diagnosis for inherited cardiac arrhythmias.
J Nippon Med Sch,
2014
, 81 (203-10).
265
Zimmer T
et al.
Voltage-gated sodium channels in the mammalian heart.
Glob Cardiol Sci Pract,
2014
, 2014 (449-63).
266
Gosselin-Badaroudine P
et al.
Nav 1.5 mutations linked to dilated cardiomyopathy phenotypes: Is the gating pore current the missing link?
Channels (Austin),
2014
, 8 (90-4).
267
Guo X
et al.
Discovery of aroyl piperazine derivatives as IKr & IKs dual inhibitors for cardiac arrhythmia treatment.
Med Chem,
2014
, 10 (497-505).
268
Barro-Soria R
et al.
KCNE1 divides the voltage sensor movement in KCNQ1/KCNE1 channels into two steps.
Nat Commun,
2014
, 5 (3750).
269
Bari V
et al.
Multiscale complexity analysis of the cardiac control identifies asymptomatic and symptomatic patients in long QT syndrome type 1.
PLoS ONE,
2014
, 9 (e93808).
270
Antzelevitch C
et al.
The role of late I Na in development of cardiac arrhythmias.
Handb Exp Pharmacol,
2014
, 221 (137-68).
271
Wu C
et al.
Kcnq1-5 (Kv7.1-5) potassium channel expression in the adult zebrafish.
BMC Physiol.,
2014
, 14 (1).
272
Winbo A
et al.
Phenotype, origin and estimated prevalence of a common long QT syndrome mutation: a clinical, genealogical and molecular genetics study including Swedish R518X/KCNQ1 families.
BMC Cardiovasc Disord,
2014
, 14 (22).
273
Kim SK
et al.
Synergistic restoring effects of isoproterenol and magnesium on KCNQ1-inhibited bradycardia cell models cultured in microelectrode array.
Cardiology,
2014
, 128 (15-24).
274
Fan Z
et al.
Efficient gene targeting in golden Syrian hamsters by the CRISPR/Cas9 system.
PLoS ONE,
2014
, 9 (e109755).
275
Nakajo K
et al.
Steric hindrance between S4 and S5 of the KCNQ1/KCNE1 channel hampers pore opening.
Nat Commun,
2014
, 5 (4100).
276
Mizusawa Y
et al.
Genetic and clinical advances in congenital long QT syndrome.
Circ. J.,
2014
, 78 (2827-33).
277
Coyan FC
et al.
A long QT mutation substitutes cholesterol for phosphatidylinositol-4,5-bisphosphate in KCNQ1 channel regulation.
PLoS ONE,
2014
, 9 (e93255).
278
Anderson CL
et al.
Large-scale mutational analysis of Kv11.1 reveals molecular insights into type 2 long QT syndrome.
Nat Commun,
2014
, 5 (5535).
279
Schweigmann U
et al.
Elevated heart rate triggers action potential alternans and sudden death. translational study of a homozygous KCNH2 mutation.
PLoS ONE,
2014
, 9 (e103150).
280
Liu L
et al.
Chromanol 293B, an inhibitor of KCNQ1 channels, enhances glucose-stimulated insulin secretion and increases glucagon-like peptide-1 level in mice.
Islets,
2014
, 6 (e962386).
281
Strutz-Seebohm N
et al.
Klotho: a new trafficking modifier of Kv7.1/KCNE1 channels.
Channels (Austin),
2014
, 8 (285).
282
Bazzi MD
et al.
Association between FTO, MC4R, SLC30A8, and KCNQ1 gene variants and type 2 diabetes in Saudi population.
Genet. Mol. Res.,
2014
, 13 (10194-203).
283
Almilaji A
et al.
Upregulation of KCNQ1/KCNE1 K+ channels by Klotho.
Channels (Austin),
2014
, 8 (222-9).
284
Ertugrul I
et al.
Follow up of a family with asymptomatic compound long QT syndrome mutations.
Genet. Couns.,
2014
, 25 (399-403).
285
Blom SM
et al.
From pan-reactive KV7 channel opener to subtype selective opener/inhibitor by addition of a methyl group.
PLoS ONE,
2014
, 9 (e100209).
286
Qureshi SF
et al.
Novel mutations of KCNQ1 in Long QT syndrome.
Indian Heart J,
2013 Sep-Oct
, 65 (552-60).
287
Beattie KA
et al.
Evaluation of an in silico cardiac safety assay: Using ion channel screening data to predict QT interval changes in the rabbit ventricular wedge.
J Pharmacol Toxicol Methods,
2013 Jul-Aug
, 68 (88-96).
288
Elkins RC
et al.
Variability in high-throughput ion-channel screening data and consequences for cardiac safety assessment.
J Pharmacol Toxicol Methods,
2013 Jul-Aug
, 68 (112-22).
289
Lang F
et al.
Serum and glucocorticoid inducible kinase, metabolic syndrome, inflammation, and tumor growth.
Hormones (Athens),
2013 Apr-Jun
, 12 (160-71).
290
Svalø J
et al.
Bladder contractility is modulated by Kv7 channels in pig detrusor.
Eur. J. Pharmacol.,
2013
Sep
5
, 715 (312-20).
291
Haugaa KH
et al.
Abnormal electroencephalograms in patients with long QT syndrome.
Heart Rhythm,
2013
Sep
27
, ().
292
Wu M
et al.
Insulin suppresses IKs (KCNQ1/KCNE1) currents, which require β-subunit KCNE1.
Pflugers Arch.,
2013
Sep
26
, ().
293
Diamant UB
et al.
Electrophysiological Phenotype in the LQTS Mutations Y111C and R518X in the KCNQ1 Gene.
J. Appl. Physiol.,
2013
Sep
19
, ().
294
Haitin Y
et al.
The structural mechanism of KCNH-channel regulation by the eag domain.
Nature,
2013
Sep
19
, 501 (444-8).
295
Cordeiro JM
et al.
Developmental changes in expression and biophysics of ion channels in the canine ventricle.
J. Mol. Cell. Cardiol.,
2013
Sep
10
, 64C (79-89).
296
Hedley PL
et al.
The Role of CAV3 in Long QT Syndrome: Clinical and Functional Assessment of a Caveolin-3/Kv11.1 Double Heterozygote Versus Caveolin-3 Single Heterozygote.
Circ Cardiovasc Genet,
2013
Sep
10
, ().
297
Liu C
et al.
Postmortem molecular analysis of KCNQ1, KCNH2, KCNE1 and KCNE2 genes in sudden unexplained nocturnal death syndrome in the Chinese Han population.
Forensic Sci. Int.,
2013
Sep
10
, 231 (82-7).
298
Remme CA
Cardiac sodium channelopathy associated with SCN5A mutations: electrophysiological, molecular and genetic aspects.
J. Physiol. (Lond.),
2013
Sep
1
, 591 (4099-116).
299
Wang H
et al.
Common variants in KCNQ1 confer increased risk of type 2 diabetes and contribute to the diabetic epidemic in East Asians: a replication and meta-analysis.
Ann. Hum. Genet.,
2013
Sep
, 77 (380-91).
300
Laksman ZW
et al.
Mutation location effect on severity of phenotype during exercise testing in type 1 long-QT syndrome: impact of transmembrane and C-loop location.
J. Cardiovasc. Electrophysiol.,
2013
Sep
, 24 (1015-20).
301
Nawathe PA
et al.
An LQTS6 MiRP1 mutation suppresses pacemaker current and is associated with sinus bradycardia.
J. Cardiovasc. Electrophysiol.,
2013
Sep
, 24 (1021-7).
302
Park JK
et al.
Single Nucleotide Deletion Mutation of KCNH2 Gene is Responsible for LQT Syndrome in a 3-Generation Korean Family.
J. Korean Med. Sci.,
2013
Sep
, 28 (1388-93).
303
Yang H
et al.
Compromised potassium recycling in the cochlea contributes to conservation of endocochlear potential in a mouse model of age-related hearing loss.
Neurosci. Lett.,
2013
Oct
25
, 555 (97-101).
304
Kimoto K
et al.
Characterization of a novel mutant KCNQ1 channel subunit lacking a large part of the C-terminal domain.
Biochem. Biophys. Res. Commun.,
2013
Oct
18
, 440 (283-8).
305
Olesen MS
et al.
Very early onset lone atrial fibrillation patients have a high prevalence of rare variants in genes previously associated with atrial fibrillation.
Heart Rhythm,
2013
Oct
18
, ().
306
Ma D
et al.
Modeling type 3 long QT syndrome with cardiomyocytes derived from patient-specific induced pluripotent stem cells.
Int. J. Cardiol.,
2013
Oct
15
, 168 (5277-86).
307
Meng J
et al.
Compound ICA-105574 prevents arrhythmias induced by cardiac delayed repolarization.
Eur. J. Pharmacol.,
2013
Oct
15
, 718 (87-97).
308
Hasegawa K
et al.
A Novel KCNQ1 Missense Mutation Identified in a Patient with Juvenile-Onset Atrial Fibrillation Causes Constitutively Open IKs Channels.
Heart Rhythm,
2013
Oct
1
, ().
309
Campbell CM
et al.
Selective targeting of gain-of-function KCNQ1 mutations predisposing to atrial fibrillation.
Circ Arrhythm Electrophysiol,
2013
Oct
, 6 (960-6).
310
Andreasen L
et al.
Genetic Modifier of the QTc Interval Associated With Early-Onset Atrial Fibrillation.
Can J Cardiol,
2013
Oct
, 29 (1234-40).
311
Veerman CC
et al.
Slow delayed rectifier potassium current blockade contributes importantly to drug-induced long QT syndrome.
Circ Arrhythm Electrophysiol,
2013
Oct
, 6 (1002-9).
312
Cuneo BF
et al.
Arrhythmia phenotype during fetal life suggests long-QT syndrome genotype: risk stratification of perinatal long-QT syndrome.
Circ Arrhythm Electrophysiol,
2013
Oct
, 6 (946-51).
313
Giudicessi JR
et al.
Genotype- and Phenotype-Guided Management of Congenital Long QT Syndrome.
Curr Probl Cardiol,
2013
Oct
, 38 (417-455).
314
Wang F
et al.
The phenotype characteristics of type 13 long QT syndrome with mutation in KCNJ5 (Kir3.4-G387R).
Heart Rhythm,
2013
Oct
, 10 (1500-6).
315
Yu W
et al.
Association between KCNQ1 genetic variants and QT interval in a Chinese population.
Diabet. Med.,
2013
Oct
, 30 (1225-9).
316
Lee YS
et al.
Long QT syndrome: a Korean single center study.
J. Korean Med. Sci.,
2013
Oct
, 28 (1454-60).
317
Hummel YM
et al.
Ventricular dysfunction in a family with long QT syndrome type 3.
Europace,
2013
Oct
, 15 (1516-21).
318
Hihara T
et al.
Probucol and the cholesterol synthesis inhibitors simvastatin and triparanol regulate I ks channel function differently.
Hum Exp Toxicol,
2013
Oct
, 32 (1028-37).
319
Bodi I
et al.
Differential effects of the β-adrenoceptor blockers carvedilol and metoprolol on SQT1- and SQT2-mutant channels.
J. Cardiovasc. Electrophysiol.,
2013
Oct
, 24 (1163-71).
320
Kamei S
et al.
Molecular analysis of potassium ion channel genes in sudden death cases among patients administered psychotropic drug therapy: are polymorphisms in LQT genes a potential risk factor?
J. Hum. Genet.,
2013
Nov
28
, ().
321
Li Y
et al.
Intracellular ATP binding is required to activate the slowly activating K+ channel I(Ks).
Proc. Natl. Acad. Sci. U.S.A.,
2013
Nov
19
, 110 (18922-7).
322
Stump MR
et al.
LQT2 nonsense mutations generate trafficking defective NH2-terminally truncated channels by the reinitiation of translation.
Am. J. Physiol. Heart Circ. Physiol.,
2013
Nov
1
, 305 (H1397-404).
323
Smith JL
et al.
Pharmacological correction of long QT-linked mutations in KCNH2 (hERG) increases the trafficking of Kv11.1 channels stored in the transitional endoplasmic reticulum.
Am. J. Physiol., Cell Physiol.,
2013
Nov
1
, 305 (C919-30).
324
Di Cori A
et al.
[Pro-arrhythmic effect of cardiac memory in a patient with long QT syndrome].
G Ital Cardiol (Rome),
2013
Nov
, 14 (746-9).
325
Wan J
et al.
A novel tetranucleotide repeat polymorphism within KCNQ1OT1 confers risk for hepatocellular carcinoma.
DNA Cell Biol.,
2013
Nov
, 32 (628-34).
326
Szlachcic A
et al.
New satiety hormone nesfatin-1 protects gastric mucosa against stress-induced injury: mechanistic roles of prostaglandins, nitric oxide, sensory nerves and vanilloid receptors.
Peptides,
2013
Nov
, 49 (9-20).
327
Zhang X
et al.
KCNQ and KCNE potassium channel subunit expression in bovine retinal pigment epithelium.
Exp. Eye Res.,
2013
Nov
, 116 (424-32).
328
Zhou P
et al.
Phosphatidylinositol 4,5-bisphosphate alters pharmacological selectivity for epilepsy-causing KCNQ potassium channels.
Proc. Natl. Acad. Sci. U.S.A.,
2013
May
21
, 110 (8726-31).
329
Yu H
et al.
Dynamic subunit stoichiometry confers a progressive continuum of pharmacological sensitivity by KCNQ potassium channels.
Proc. Natl. Acad. Sci. U.S.A.,
2013
May
21
, 110 (8732-7).
330
Boczek NJ
et al.
Exome Sequencing and Systems Biology Converge to Identify Novel Mutations in the L-Type Calcium Channel, CACNA1C, Linked to Autosomal Dominant Long QT Syndrome.
Circ Cardiovasc Genet,
2013
May
15
, ().
331
Liu J
et al.
Meta-analysis of the effect of KCNQ1 gene polymorphism on the risk of type 2 diabetes.
Mol. Biol. Rep.,
2013
May
, 40 (3557-67).
332
McBride CM
et al.
Mechanistic basis for type 2 long QT syndrome caused by KCNH2 mutations that disrupt conserved arginine residues in the voltage sensor.
J. Membr. Biol.,
2013
May
, 246 (355-64).
333
An HS
et al.
Sudden cardiac arrest during anesthesia in a 30-month-old boy with syndactyly: a case of genetically proven Timothy syndrome.
J. Korean Med. Sci.,
2013
May
, 28 (788-91).
334
Aziz PF
et al.
Do LQTS gene single nucleotide polymorphisms alter QTc intervals at rest and during exercise stress testing?
Ann Noninvasive Electrocardiol,
2013
May
, 18 (288-93).
335
Khoueiry R
et al.
Abnormal methylation of KCNQ1OT1 and differential methylation of H19 imprinting control regions in human ICSI embryos.
Zygote,
2013
May
, 21 (129-38).
336
Bartos DC
et al.
A KCNQ1 mutation causes a high penetrance for familial atrial fibrillation.
J. Cardiovasc. Electrophysiol.,
2013
May
, 24 (562-9).
337
Hoosien M
et al.
Dysfunctional potassium channel subunit interaction as a novel mechanism of long QT syndrome.
Heart Rhythm,
2013
May
, 10 (728-37).
338
Matsa E
et al.
Allele-specific RNA interference rescues the long-QT syndrome phenotype in human-induced pluripotency stem cell cardiomyocytes.
Eur. Heart J.,
2013
Mar
6
, ().
339
Werry D
et al.
Single-channel basis for the slow activation of the repolarizing cardiac potassium current, I(Ks).
Proc. Natl. Acad. Sci. U.S.A.,
2013
Mar
12
, 110 (E996-1005).
340
Chon SJ
et al.
Association of variants in PPARγ², IGF2BP2, and KCNQ1 with a susceptibility to gestational diabetes mellitus in a Korean population.
Yonsei Med. J.,
2013
Mar
1
, 54 (352-7).
341
Babcock JJ
et al.
hERG channel function: beyond long QT.
Acta Pharmacol. Sin.,
2013
Mar
, 34 (329-35).
342
Travers ME
et al.
Insights into the molecular mechanism for type 2 diabetes susceptibility at the KCNQ1 locus from temporal changes in imprinting status in human islets.
Diabetes,
2013
Mar
, 62 (987-92).
343
Jehle J
et al.
Mechanisms of zolpidem-induced long QT syndrome: acute inhibition of recombinant hERG K(+) channels and action potential prolongation in human cardiomyocytes derived from induced pluripotent stem cells.
Br. J. Pharmacol.,
2013
Mar
, 168 (1215-29).
344
Lorente-Cánovas B
et al.
Mice deficient in H+-ATPase a4 subunit have severe hearing impairment associated with enlarged endolymphatic compartments within the inner ear.
Dis Model Mech,
2013
Mar
, 6 (434-42).
345
Lundby A
et al.
In vivo phosphoproteomics analysis reveals the cardiac targets of β-adrenergic receptor signaling.
Sci Signal,
2013
Jun
4
, 6 (rs11).
346
Steinke K
et al.
Coxsackievirus B3 modulates cardiac ion channels.
FASEB J.,
2013
Jun
27
, ().
347
Vaidyanathan R
et al.
The Interaction of Caveolin 3 Protein with the Potassium Inward Rectifier Channel Kir2.1: PHYSIOLOGY AND PATHOLOGY RELATED TO LONG QT SYNDROME 9 (LQT9).
J. Biol. Chem.,
2013
Jun
14
, 288 (17472-80).
348
De La Rosa AJ
et al.
Functional suppression of Kcnq1 leads to early sodium channel remodelling and cardiac conduction system dysmorphogenesis.
Cardiovasc. Res.,
2013
Jun
1
, 98 (504-14).
349
Li J
et al.
Anti-KCNQ1 K⁺ channel autoantibodies increase IKs current and are associated with QT interval shortening in dilated cardiomyopathy.
Cardiovasc. Res.,
2013
Jun
1
, 98 (496-503).
350
Heijman J
et al.
KCNQ1 autoantibodies: another way to regulate IKs.
Cardiovasc. Res.,
2013
Jun
1
, 98 (329-31).
351
Rapetti-Mauss R
et al.
Oestrogen promotes KCNQ1 potassium channel endocytosis and postendocytic trafficking in colonic epithelium.
J. Physiol. (Lond.),
2013
Jun
1
, 591 (2813-31).
352
Winbo A
et al.
Iron-deficiency anaemia, gastric hyperplasia, and elevated gastrin levels due to potassium channel dysfunction in the Jervell and Lange-Nielsen Syndrome.
Cardiol Young,
2013
Jun
, 23 (325-34).
353
Luo Y
et al.
Estrogen-related receptor γ (ERRγ) regulates oxygen-dependent expression of voltage-gated potassium (K+) channels and tissue kallikrein during human trophoblast differentiation.
Mol. Endocrinol.,
2013
Jun
, 27 (940-52).
354
Sachse CC
et al.
BACE1 and presenilin/γ-secretase regulate proteolytic processing of KCNE1 and 2, auxiliary subunits of voltage-gated potassium channels.
FASEB J.,
2013
Jun
, 27 (2458-67).
355
Mahida S
et al.
Genetics of congenital and drug-induced long QT syndromes: current evidence and future research perspectives.
J Interv Card Electrophysiol,
2013
Jun
, 37 (9-19).
356
Lang F
et al.
Therapeutic potential of serum and glucocorticoid inducible kinase inhibition.
Expert Opin Investig Drugs,
2013
Jun
, 22 (701-14).
357
Gravel AE
et al.
An NMR investigation of the structure, function and role of the hERG channel selectivity filter in the long QT syndrome.
Biochim. Biophys. Acta,
2013
Jun
, 1828 (1494-502).
358
Lin YD
et al.
[Association of polymorphisms of potassium voltage-gated channel, KQT-like subfamily, member 1 and type 2 diabetes in Jiangsu province, China].
Zhonghua Yu Fang Yi Xue Za Zhi,
2013
Jun
, 47 (538-41).
359
Brelidze TI
et al.
Structure of the C-terminal region of an ERG channel and functional implications.
Proc. Natl. Acad. Sci. U.S.A.,
2013
Jul
9
, 110 (11648-53).
360
Amin AS
et al.
Long QT syndrome: beyond the causal mutation.
J. Physiol. (Lond.),
2013
Jul
22
, ().
361
Jost N
et al.
Ionic mechanisms limiting cardiac repolarization-reserve in humans compared to dogs.
J. Physiol. (Lond.),
2013
Jul
22
, ().
362
Zaydman MA
et al.
Kv7.1 ion channels require a lipid to couple voltage sensing to pore opening.
Proc. Natl. Acad. Sci. U.S.A.,
2013
Jul
16
, ().
363
Duchatelet S
et al.
Identification of a KCNQ1 Polymorphism Acting as a Protective Modifier against Arrhythmic Risk in Long QT Syndrome.
Circ Cardiovasc Genet,
2013
Jul
15
, ().
364
Takanari H
et al.
Efficient and specific cardiac IK1 inhibition by a new pentamidine analogue.
Cardiovasc. Res.,
2013
Jul
1
, 99 (203-14).
365
Son MK
et al.
Genetic mutation in korean patients of sudden cardiac arrest as a surrogating marker of idiopathic ventricular arrhythmia.
J. Korean Med. Sci.,
2013
Jul
, 28 (1021-6).
366
Varkevisser R
et al.
Structure-activity relationships of pentamidine-affected ion channel trafficking and dofetilide mediated rescue.
Br. J. Pharmacol.,
2013
Jul
, 169 (1322-34).
367
Kaltenbach S
et al.
Beckwith-Wiedemann syndrome and long QT syndrome due to familial-balanced translocation t(11;17)(p15.5;q21.3) involving the KCNQ1 gene.
Clin. Genet.,
2013
Jul
, 84 (78-81).
368
Telezhkin V
et al.
A basic residue in the proximal C-terminus is necessary for efficient activation of the M-channel subunit Kv7.2 by PI(4,5)P₂.
Pflugers Arch.,
2013
Jul
, 465 (945-53).
369
Lieve KV
et al.
Results of genetic testing in 855 consecutive unrelated patients referred for long QT syndrome in a clinical laboratory.
Genet Test Mol Biomarkers,
2013
Jul
, 17 (553-61).
370
Jou CJ
et al.
An In Vivo Cardiac Assay to Determine the Functional Consequences of Putative Long QT Syndrome Mutations.
Circ. Res.,
2013
Jan
9
, ().
371
Nguyen N
et al.
Independent trafficking of the KCNQ1 K+ channel and H+-K+-ATPase in gastric parietal cells from mice.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2013
Jan
15
, 304 (G157-66).
372
Zhang Y
et al.
Arsenic trioxide-induced hERG K(+) channel deficiency can be rescued by matrine and oxymatrine through up-regulating transcription factor Sp1 expression.
Biochem. Pharmacol.,
2013
Jan
1
, 85 (59-68).
373
Feng MJ
et al.
[KCNQ1 mutation in patients with lone atrial fibrillation].
Zhonghua Xin Xue Guan Bing Za Zhi,
2013
Jan
, 41 (8-12).
374
Nakashima K
et al.
A left ventricular noncompaction in a patient with long QT syndrome caused by a KCNQ1 mutation: a case report.
Heart Vessels,
2013
Jan
, 28 (126-9).
375
Lu X
et al.
RNA interference targeting E637K mutation rescues hERG channel currents and restores its kinetic properties.
Heart Rhythm,
2013
Jan
, 10 (128-36).
376
Liu L
et al.
A novel mutation in the transmembrane nonpore region of the KCNH2 gene causes severe clinical manifestations of long QT syndrome.
Heart Rhythm,
2013
Jan
, 10 (61-7).
377
Schneider HE
et al.
Left cardiac sympathetic denervation for the management of life-threatening ventricular tachyarrhythmias in young patients with catecholaminergic polymorphic ventricular tachycardia and long QT syndrome.
Clin Res Cardiol,
2013
Jan
, 102 (33-42).
378
Giudicessi JR
et al.
Determinants of incomplete penetrance and variable expressivity in heritable cardiac arrhythmia syndromes.
Transl Res,
2013
Jan
, 161 (1-14).
379
Terrenoire C
et al.
Induced pluripotent stem cells used to reveal drug actions in a long QT syndrome family with complex genetics.
J. Gen. Physiol.,
2013
Jan
, 141 (61-72).
380
Abbott GW
KCNE genetics and pharmacogenomics in cardiac arrhythmias: much ado about nothing?
Expert Rev Clin Pharmacol,
2013
Jan
, 6 (49-60).
381
Zhang A
et al.
L539 fs/47, a truncated mutation of human ether-a-go-go-related gene (hERG), decreases hERG ion channel currents in HEK 293 cells.
Clin. Exp. Pharmacol. Physiol.,
2013
Jan
, 40 (28-36).
382
Lang F
et al.
Regulation of ion channels by the serum- and glucocorticoid-inducible kinase SGK1.
FASEB J.,
2013
Jan
, 27 (3-12).
383
Bostan O
et al.
Jervell and Lange-Nielsen Syndrome: Homozygous Missense Mutation of KCNQ1 in a Turkish Family.
Pediatr Cardiol,
2013
Feb
12
, ().
384
Ruscic KJ
et al.
IKs channels open slowly because KCNE1 accessory subunits slow the movement of S4 voltage sensors in KCNQ1 pore-forming subunits.
Proc. Natl. Acad. Sci. U.S.A.,
2013
Feb
12
, 110 (E559-66).
385
Yang T
et al.
An Allosteric Mechanism for Drug Block of the Human Cardiac Potassium Channel KCNQ1.
Mol. Pharmacol.,
2013
Feb
, 83 (481-9).
386
Melnick M
et al.
An in vitro mouse model of congenital cytomegalovirus-induced pathogenesis of the inner ear cochlea.
Birth Defects Res. Part A Clin. Mol. Teratol.,
2013
Feb
, 97 (69-78).
387
De Crescenzo A
et al.
Paternal deletion of the 11p15.5 centromeric-imprinting control region is associated with alteration of imprinted gene expression and recurrent severe intrauterine growth restriction.
J. Med. Genet.,
2013
Feb
, 50 (99-103).
388
Siraskar B
et al.
Downregulation of the renal outer medullary K(+) channel ROMK by the AMP-activated protein kinase.
Pflugers Arch.,
2013
Feb
, 465 (233-45).
389
Ware JS
et al.
Next generation diagnostics in inherited arrhythmia syndromes : a comparison of two approaches.
J Cardiovasc Transl Res,
2013
Feb
, 6 (94-103).
390
Wang Y
et al.
[Ca2+]i elevation and oxidative stress induce KCNQ1 protein translocation from the cytosol to the cell surface and increase slow delayed rectifier (IKs) in cardiac myocytes.
J. Biol. Chem.,
2013
Dec
6
, 288 (35358-71).
391
Nguyen HL
et al.
Andersen-Tawil syndrome: clinical and molecular aspects.
Int. J. Cardiol.,
2013
Dec
5
, 170 (1-16).
392
Xu Y
et al.
Building KCNQ1/KCNE1 channel models and probing their interactions by molecular-dynamics simulations.
Biophys. J.,
2013
Dec
3
, 105 (2461-73).
393
Seebohm G
A complex partnership: KCNQ1 and KCNE1.
Biophys. J.,
2013
Dec
3
, 105 (2437-8).
394
Andersen MN
et al.
A Phosphoinositide 3-Kinase (PI3K)-serum- and glucocorticoid-inducible Kinase 1 (SGK1) Pathway Promotes Kv7.1 Channel Surface Expression by Inhibiting Nedd4-2 Protein.
J. Biol. Chem.,
2013
Dec
27
, 288 (36841-54).
395
Bellin M
et al.
Isogenic human pluripotent stem cell pairs reveal the role of a KCNH2 mutation in long-QT syndrome.
EMBO J.,
2013
Dec
11
, 32 (3161-75).
396
Uesugi M
et al.
Low-density plating is sufficient to induce cardiac hypertrophy and electrical remodeling in highly purified human iPS cell-derived cardiomyocytes.
J Pharmacol Toxicol Methods,
2013
Dec
1
, ().
397
Khanamiri S
et al.
Contribution of Kv7 channels to basal coronary flow and active response to ischemia.
Hypertension,
2013
Dec
, 62 (1090-7).
398
Hintsa T
et al.
Work stress and the long QT syndrome: high job strain and effort-reward imbalance at work associated with arrhythmic risk in the long QT syndrome.
J. Occup. Environ. Med.,
2013
Dec
, 55 (1387-93).
399
Apaja PM
et al.
Ubiquitination-dependent quality control of hERG K+ channel with acquired and inherited conformational defect at the plasma membrane.
Mol. Biol. Cell,
2013
Dec
, 24 (3787-804).
400
Kato K
et al.
Cardiac Channelopathies Associated with Infantile Fatal Ventricular Arrhythmias: From the Cradle to the Bench.
J. Cardiovasc. Electrophysiol.,
2013
Aug
26
, ().
401
Than BL
et al.
The role of KCNQ1 in mouse and human gastrointestinal cancers.
Oncogene,
2013
Aug
26
, ().
402
Hayashi M
et al.
Molecular basis of potassium channels in pancreatic duct epithelial cells.
Channels (Austin),
2013
Aug
20
, 7 ().
403
Li Y
et al.
MicroRNA-1/133 targeted dysfunction of potassium channels KCNE1 and KCNQ1 in human cardiac progenitor cells with simulated hyperglycemia.
Int. J. Cardiol.,
2013
Aug
10
, 167 (1076-8).
404
Gao Y
et al.
A novel deletion-frameshift mutation in the S1 region of HERG gene in a Chinese family with long QT syndrome.
Chin. Med. J.,
2013
Aug
, 126 (3093-6).
405
Cheng J
et al.
Caveolin-3 suppresses late sodium current by inhibiting nNOS-dependent S-nitrosylation of SCN5A.
J. Mol. Cell. Cardiol.,
2013
Aug
, 61 (102-10).
406
Lee HC
et al.
Modulation of KCNQ1 alternative splicing regulates cardiac IKs and action potential repolarization.
Heart Rhythm,
2013
Aug
, 10 (1220-8).
407
Thomsen MB
Kv7.1 isoform gradients in the heart: new potential approach to alter repolarization reserve.
Heart Rhythm,
2013
Aug
, 10 (1229-30).
408
Maslarova A
et al.
Increased susceptibility to acetylcholine in the entorhinal cortex of pilocarpine-treated rats involves alterations in KCNQ channels.
Neurobiol. Dis.,
2013
Aug
, 56 (14-24).
409
Hanson RL
et al.
Strong parent-of-origin effects in the association of KCNQ1 variants with type 2 diabetes in American Indians.
Diabetes,
2013
Aug
, 62 (2984-91).
410
Tsai WC
et al.
Ablation of the androgen receptor gene modulates atrial electrophysiology and arrhythmogenesis with calcium protein dysregulation.
Endocrinology,
2013
Aug
, 154 (2833-42).
411
Partemi S
et al.
Loss-of-function KCNH2 mutation in a family with long QT syndrome, epilepsy, and sudden death.
Epilepsia,
2013
Aug
, 54 (e112-6).
412
Arslan Yildiz A
et al.
Biomimetic membrane platform containing hERG potassium channel and its application to drug screening.
Analyst,
2013
Apr
7
, 138 (2007-12).
413
de Pascual R
et al.
Lower density of L-type and higher density of P/Q-type of calcium channels in chromaffin cells of hypertensive, compared with normotensive rats.
Eur. J. Pharmacol.,
2013
Apr
15
, 706 (25-35).
414
Crotti L
et al.
Long QT syndrome-associated mutations in intrauterine fetal death.
JAMA,
2013
Apr
10
, 309 (1473-82).
415
Hu RM
et al.
Digenic inheritance novel mutations in SCN5a and SNTA1 increase late I(Na) contributing to LQT syndrome.
Am. J. Physiol. Heart Circ. Physiol.,
2013
Apr
1
, 304 (H994-H1001).
416
Gao M
et al.
An altered expression of genes involved in the regulation of ion channels in atrial myocytes is correlated with the risk of atrial fibrillation in patients with heart failure.
Exp Ther Med,
2013
Apr
, 5 (1239-1243).
417
Shinwari ZM
et al.
Identification of a novel KCNQ1 mutation in a large Saudi family with long QT syndrome: clinical consequences and preventive implications.
Clin. Genet.,
2013
Apr
, 83 (370-4).
418
Giudicessi JR
et al.
Prevalence and potential genetic determinants of sensorineural deafness in KCNQ1 homozygosity and compound heterozygosity.
Circ Cardiovasc Genet,
2013
Apr
, 6 (193-200).
419
Netchine I
et al.
Epigenetic anomalies in childhood growth disorders.
Nestle Nutr Inst Workshop Ser,
2013
, 71 (65-73).
420
Trolle C
et al.
Long QT interval in Turner syndrome--a high prevalence of LQTS gene mutations.
PLoS ONE,
2013
, 8 (e69614).
421
Theilade J
et al.
Cascade screening in families with inherited cardiac diseases driven by cardiologists: feasibility and nationwide outcome in long QT syndrome.
Cardiology,
2013
, 126 (131-7).
422
Afeli SA
et al.
Molecular expression and pharmacological evidence for a functional role of kv7 channel subtypes in Guinea pig urinary bladder smooth muscle.
PLoS ONE,
2013
, 8 (e75875).
423
Strutz-Seebohm N
et al.
A Common Structural Component for β-Subunit Mediated Modulation of Slow Inactivation in Different KV Channels.
Cell. Physiol. Biochem.,
2013
, 31 (968-80).
424
Hua Z
et al.
Chemical derivatization and purification of peptide-toxins for probing ion channel complexes.
Methods Mol. Biol.,
2013
, 995 (19-30).
425
Hsiao PY
et al.
Gene mutations in cardiac arrhythmias: a review of recent evidence in ion channelopathies.
Appl Clin Genet,
2013
, 6 (1-13).
426
Li X
et al.
Endolymphatic Na(+) and K(+) Concentrations during Cochlear Growth and Enlargement in Mice Lacking Slc26a4/pendrin.
PLoS ONE,
2013
, 8 (e65977).
427
Vyas V
et al.
The investigation of sudden arrhythmic death syndrome (SADS)-the current approach to family screening and the future role of genomics and stem cell technology.
Front Physiol,
2013
, 4 (199).
428
Shimizu W
Update of diagnosis and management of inherited cardiac arrhythmias.
Circ. J.,
2013
, 77 (2867-72).
429
Fan Y
et al.
Genistein ameliorates adverse cardiac effects induced by arsenic trioxide through preventing cardiomyocytes apoptosis.
Cell. Physiol. Biochem.,
2013
, 31 (80-91).
430
Chu HM
et al.
Polymorphisms but not mutations of the KCNQ1 gene are associated with lone atrial fibrillation in the Chinese Han population.
ScientificWorldJournal,
2013
, 2013 (373454).
431
Moric-Janiszewska E
et al.
Quantitative PCR as an Alternative in the Diagnosis of Long-QT Syndrome.
Biomed Res Int,
2013
, 2013 (418604).
432
Stolk L
et al.
Epigenetic profiles in children with a neural tube defect; a case-control study in two populations.
PLoS ONE,
2013
, 8 (e78462).
433
Fatima A
et al.
The disease-specific phenotype in cardiomyocytes derived from induced pluripotent stem cells of two long QT syndrome type 3 patients.
PLoS ONE,
2013
, 8 (e83005).
434
Kosenko A
et al.
A change in configuration of the calmodulin-KCNQ channel complex underlies Ca2+-dependent modulation of KCNQ channel activity.
PLoS ONE,
2013
, 8 (e82290).
435
Shu L
et al.
Modulation of HERG K+ channels by chronic exposure to activators and inhibitors of PKA and PKC: actions independent of PKA and PKC phosphorylation.
Cell. Physiol. Biochem.,
2013
, 32 (1830-44).
436
Uziębło-Życzkowska B
et al.
Congenital long QT syndrome of particularly malignant course connected with so far unknown mutation in the sodium channel SCN5A gene.
Cardiol J,
2013
, 20 (78-82).
437
Pérez Riera AR
et al.
Congenital short QT syndrome: landmarks of the newest arrhythmogenic cardiac channelopathy.
Cardiol J,
2013
, 20 (464-71).
438
Okata S
et al.
The generation of induced pluripotent stem cells from a patient with KCNH2 G603D, without LQT2 disease associated symptom.
J. Med. Dent. Sci.,
2013
, 60 (17-22).
439
Ferrer T
et al.
Tamoxifen inhibition of kv7.2/kv7.3 channels.
PLoS ONE,
2013
, 8 (e76085).
440
Andrsova I
et al.
Clinical characteristics of 30 Czech families with long QT syndrome and KCNQ1 and KCNH2 gene mutations: importance of exercise testing.
J Electrocardiol,
2012 Nov-Dec
, 45 (746-51).
441
Lin L
et al.
Drug-induced QT-interval prolongation and recurrent torsade de pointes in a child with heterotaxy syndrome and KCNE1 D85N polymorphism.
J Electrocardiol,
2012 Nov-Dec
, 45 (770-3).
442
Hekkala AM
et al.
T-wave morphology after epinephrine bolus may reveal silent long QT syndrome mutation carriers.
J Electrocardiol,
2012 Jul-Aug
, 45 (368-72).
443
Jindal HK
et al.
Proteomic analyses of transgenic LQT1 and LQT2 rabbit hearts elucidate an increase in expression and activity of energy producing enzymes.
J Proteomics,
2012
Sep
18
, 75 (5254-65).
444
Kharche S
et al.
Pro-arrhythmogenic effects of the S140G KCNQ1 mutation in human atrial fibrillation - insights from modelling.
J. Physiol. (Lond.),
2012
Sep
15
, 590 (4501-14).
445
Mattmann ME
et al.
Identification of (R)-N-(4-(4-methoxyphenyl)thiazol-2-yl)-1-tosylpiperidine-2-carboxamide, ML277, as a novel, potent and selective K(v)7.1 (KCNQ1) potassium channel activator.
Bioorg. Med. Chem. Lett.,
2012
Sep
15
, 22 (5936-41).
446
Odgerel Z
et al.
Genetic variants in potassium channels are associated with type 2 diabetes in a Mongolian population.
J Diabetes,
2012
Sep
, 4 (238-42).
447
Zumhagen S
et al.
Inherited long QT syndrome: clinical manifestation, genetic diagnostics, and therapy.
Herzschrittmacherther Elektrophysiol,
2012
Sep
, 23 (211-9).
448
Wijnen M
et al.
KCNQ1OT1 hypomethylation: a novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?
Pediatr Blood Cancer,
2012
Sep
, 59 (565-6).
449
Korostowski L
et al.
The Kcnq1ot1 long non-coding RNA affects chromatin conformation and expression of Kcnq1, but does not regulate its imprinting in the developing heart.
PLoS Genet.,
2012
Sep
, 8 (e1002956).
450
Lim XL
et al.
KCNQ1 SNPS and susceptibility to diabetic nephropathy in East Asians with type 2 diabetes.
Diabetologia,
2012
Sep
, 55 (2402-6).
451
Peng HH
et al.
DNA methylation patterns of imprinting centers for H19, SNRPN, and KCNQ1OT1 in single-cell clones of human amniotic fluid mesenchymal stem cell.
Taiwan J Obstet Gynecol,
2012
Sep
, 51 (342-9).
452
Meisel E
et al.
KCNQ1 channels do not undergo concerted but sequential gating transitions in both the absence and the presence of KCNE1 protein.
J. Biol. Chem.,
2012
Oct
5
, 287 (34212-24).
453
Haugaa KH
et al.
Subclinical Cardiomyopathy and Long QT Syndrome: An Echocardiographic Observation.
Congenit Heart Dis,
2012
Oct
24
, ().
454
Abderemane-Ali F
et al.
Dual effect of phosphatidyl (4,5)-bisphosphate PIP2 on Shaker K+ channels.
J. Biol. Chem.,
2012
Oct
19
, 287 (36158-67).
455
Song W
et al.
The human Nav1.5 F1486 deletion associated with long QT syndrome leads to impaired sodium channel inactivation and reduced lidocaine sensitivity.
J. Physiol. (Lond.),
2012
Oct
15
, 590 (5123-39).
456
Giudicessi JR
et al.
Phylogenetic and physicochemical analyses enhance the classification of rare nonsynonymous single nucleotide variants in type 1 and 2 long-QT syndrome.
Circ Cardiovasc Genet,
2012
Oct
1
, 5 (519-28).
457
Chu W
et al.
Arsenic-induced interstitial myocardial fibrosis reveals a new insight into drug-induced long QT syndrome.
Cardiovasc. Res.,
2012
Oct
1
, 96 (90-8).
458
Föller M
et al.
AMP-activated protein kinase in BK-channel regulation and protection against hearing loss following acoustic overstimulation.
FASEB J.,
2012
Oct
, 26 (4243-53).
459
Winkel BG
et al.
The prevalence of mutations in KCNQ1, KCNH2, and SCN5A in an unselected national cohort of young sudden unexplained death cases.
J. Cardiovasc. Electrophysiol.,
2012
Oct
, 23 (1092-8).
460
Mannino GC
et al.
Individualized therapy for type 2 diabetes: clinical implications of pharmacogenetic data.
Mol Diagn Ther,
2012
Oct
, 16 (285-302).
461
Yu W
et al.
Association between KCNQ1 genetic variants and obesity in Chinese patients with type 2 diabetes.
Diabetologia,
2012
Oct
, 55 (2655-9).
462
Zhao QH
et al.
[KCNQ1, KCNH2, KCNE1 and KCNE2 potassium channels gene variants in sudden manhood death syndrome].
Fa Yi Xue Za Zhi,
2012
Oct
, 28 (337-41, 346).
463
Hoefen R
et al.
In silico cardiac risk assessment in patients with long QT syndrome: type 1: clinical predictability of cardiac models.
J. Am. Coll. Cardiol.,
2012
Nov
20
, 60 (2182-91).
464
Burgess DE
et al.
High-risk long QT syndrome mutations in the Kv7.1 (KCNQ1) pore disrupt the molecular basis for rapid K(+) permeation.
Biochemistry,
2012
Nov
13
, 51 (9076-85).
465
Duehmke RM
et al.
Altered re-excitation thresholds and conduction of extrasystolic action potentials contribute to arrhythmogenicity in murine models of long QT syndrome.
Acta Physiol (Oxf),
2012
Nov
, 206 (164-77).
466
Edelmann J
et al.
Long QT syndrome mutation detection by SNaPshot technique.
Int. J. Legal Med.,
2012
Nov
, 126 (969-73).
467
Stump MR
et al.
Early LQT2 nonsense mutation generates N-terminally truncated hERG channels with altered gating properties by the reinitiation of translation.
J. Mol. Cell. Cardiol.,
2012
Nov
, 53 (725-33).
468
Abriel H
Cardiac Sodium Channel Nav1.5 Mechanosensitivity is Inhibited by Ranolazine.
,
2012
May
7
, ().
469
Donovan AJ
et al.
LQT2 Mutation on the Kv11.1 Ion Channel Inhibits Current Activity by Ablating a PKCα Consensus Site.
,
2012
May
31
, ().
470
Du YM
et al.
18β-Glycyrrhetinic acid preferentially blocks late Na current generated by ΔKPQ Nav1.5 channels.
,
2012
May
21
, ().
472
Osteen JD
et al.
Allosteric gating mechanism underlies the flexible gating of KCNQ1 potassium channels.
Proc. Natl. Acad. Sci. U.S.A.,
2012
May
1
, 109 (7103-8).
473
Harmer SC
et al.
Readthrough of long-QT syndrome type 1 nonsense mutations rescues function but alters the biophysical properties of the channel.
Biochem. J.,
2012
May
1
, 443 (635-42).
474
Birner C
et al.
Differential expression of potassium channels and abnormal conduction in experimental tachycardia-induced heart failure.
Naunyn Schmiedebergs Arch. Pharmacol.,
2012
May
, 385 (473-80).
475
Chen J
et al.
Binding interface of cardiac potassium channel proteins identified by hydrogen deuterium exchange of synthetic peptides.
Anal Bioanal Chem,
2012
May
, 403 (1303-9).
476
Smushkin G
et al.
Diabetes-associated common genetic variation and its association with GLP-1 concentrations and response to exogenous GLP-1.
Diabetes,
2012
May
, 61 (1082-9).
477
Kapoor S
Beyond diabetes mellitus: close pathological association of mutations of the KCNQ1 gene with other systemic disorders.
Ann. Acad. Med. Singap.,
2012
May
, 41 (233).
478
Dai XP
et al.
KCNQ1 gene polymorphisms are associated with the therapeutic efficacy of repaglinide in Chinese type 2 diabetic patients.
Clin. Exp. Pharmacol. Physiol.,
2012
May
, 39 (462-8).
479
Saint-Criq V
et al.
Estrogen regulation of epithelial ion transport: Implications in health and disease.
,
2012
Mar
5
, ().
480
481
482
Liu GX
et al.
Differential conditions for early after-depolarizations and triggered activity in cardiomyocytes derived from transgenic LQT1 and LQT2 rabbits.
J. Physiol. (Lond.),
2012
Mar
1
, 590 (1171-80).
483
Pattnaik BR
et al.
Effects of KCNQ channel modulators on the M-type potassium current in primate retinal pigment epithelium.
Am. J. Physiol., Cell Physiol.,
2012
Mar
, 302 (C821-33).
484
Moreno JD
et al.
Pathophysiology of the cardiac late Na current and its potential as a drug target.
J. Mol. Cell. Cardiol.,
2012
Mar
, 52 (608-19).
485
Besana A
et al.
Nadolol block of Nav1.5 does not explain its efficacy in the long QT syndrome.
J. Cardiovasc. Pharmacol.,
2012
Mar
, 59 (249-53).
486
Chang YC
et al.
Replication of genome-wide association signals of type 2 diabetes in Han Chinese in a prospective cohort.
Clin. Endocrinol. (Oxf),
2012
Mar
, 76 (365-72).
487
Amin AS
et al.
Variants in the 3' untranslated region of the KCNQ1-encoded Kv7.1 potassium channel modify disease severity in patients with type 1 long QT syndrome in an allele-specific manner.
Eur. Heart J.,
2012
Mar
, 33 (714-23).
488
Krzystanek K
et al.
Deubiquitylating enzyme USP2 counteracts Nedd4-2-mediated downregulation of KCNQ1 potassium channels.
Heart Rhythm,
2012
Mar
, 9 (440-8).
489
Kaddar N
et al.
Tizanidine (Zanaflex): a muscle relaxant that may prolong the QT interval by blocking IKr.
J. Cardiovasc. Pharmacol. Ther.,
2012
Mar
, 17 (102-9).
490
Gönczi M
et al.
Age-dependent changes in ion channel mRNA expression in canine cardiac tissues.
Gen. Physiol. Biophys.,
2012
Jun
, 31 (153-62).
491
Tester DJ
et al.
Cardiac channel molecular autopsy: insights from 173 consecutive cases of autopsy-negative sudden unexplained death referred for postmortem genetic testing.
Mayo Clin. Proc.,
2012
Jun
, 87 (524-39).
492
Parikh A
et al.
Ranolazine stabilizes cardiac ryanodine receptors: a novel mechanism for the suppression of early afterdepolarization and torsades de pointes in long QT type 2.
Heart Rhythm,
2012
Jun
, 9 (953-60).
493
Costa J
et al.
Combined assessment of sex- and mutation-specific information for risk stratification in type 1 long QT syndrome.
Heart Rhythm,
2012
Jun
, 9 (892-8).
494
495
Gofman Y
et al.
How Does KCNE1 Regulate the Kv7.1 Potassium Channel? Model-Structure, Mutations, and Dynamics of the Kv7.1-KCNE1 Complex.
,
2012
Jul
3
, ().
496
Kulzer M
et al.
Inhibition of cardiac Kir2.1-2.3 channels by beta3 adrenoreceptor antagonist SR 59230A.
Biochem. Biophys. Res. Commun.,
2012
Jul
27
, 424 (315-20).
497
Egashira T
et al.
Disease characterization using LQTS-specific induced pluripotent stem cells.
,
2012
Jul
24
, ().
498
Zhang H
et al.
Modulation of hERG potassium channel gating normalizes action potential duration prolonged by dysfunctional KCNQ1 potassium channel.
Proc. Natl. Acad. Sci. U.S.A.,
2012
Jul
17
, 109 (11866-71).
499
Hayashi M
et al.
An intermediate-conductance Ca2+-activated K+ channel is important for secretion in pancreatic duct cells.
Am. J. Physiol., Cell Physiol.,
2012
Jul
15
, 303 (C151-9).
500
Hammami S
et al.
Purinergic signalling - a possible mechanism for KCNQ1 channel response to cell volume challenges.
,
2012
Jul
14
, ().
501
Ritchie MD
et al.
Chromosome 4q25 Variants Are Genetic Modifiers of Rare Ion Channel Mutations Associated With Familial Atrial Fibrillation.
,
2012
Jul
10
, ().
502
Crotti L
et al.
Torsades de pointes following acute myocardial infarction: evidence for a deadly link with a common genetic variant.
Heart Rhythm,
2012
Jul
, 9 (1104-12).
503
Farrugia A
et al.
Detection of genetic variation in KCNQ1 gene by high-resolution melting analysis in a prospective-based series of postmortem negative sudden death: comparison of results obtained in fresh frozen and formalin-fixed paraffin-embedded tissues.
Int. J. Legal Med.,
2012
Jul
, 126 (649-57).
504
Rosengren AH
et al.
Reduced insulin exocytosis in human pancreatic β-cells with gene variants linked to type 2 diabetes.
Diabetes,
2012
Jul
, 61 (1726-33).
505
Vandenberg JI
et al.
hERG K(+) channels: structure, function, and clinical significance.
Physiol. Rev.,
2012
Jul
, 92 (1393-478).
506
Bardou O
et al.
K+ channels regulate ENaC expression via changes in promoter activity and control fluid clearance in alveolar epithelial cells.
Biochim. Biophys. Acta,
2012
Jul
, 1818 (1682-90).
507
Gunthorpe MJ
et al.
The mechanism of action of retigabine (ezogabine), a first-in-class K(+) channel opener for the treatment of epilepsy.
,
2012
Jan
5
, ().
508
Heijman J
et al.
Dominant-negative control of cAMP-dependent IKs upregulation in human long-QT syndrome type 1.
Circ. Res.,
2012
Jan
20
, 110 (211-9).
509
Chadha PS
et al.
Pharmacological dissection of K(v) 7.1 channels in systemic and pulmonary arteries.
,
2012
Jan
17
, ().
510
Guseva N
et al.
Antisense noncoding RNA promoter regulates the timing of de novo methylation of an imprinting control region.
Dev. Biol.,
2012
Jan
15
, 361 (403-11).
511
Chiesa N
et al.
The KCNQ1OT1 imprinting control region and non-coding RNA: new properties derived from the study of Beckwith-Wiedemann syndrome and Silver-Russell syndrome cases.
Hum. Mol. Genet.,
2012
Jan
1
, 21 (10-25).
512
Andersen MN
et al.
AMP-activated protein kinase downregulates Kv7.1 cell surface expression.
Traffic,
2012
Jan
, 13 (143-56).
513
Krishnan Y
et al.
Partially dominant mutant channel defect corresponding with intermediate LQT2 phenotype.
Pacing Clin Electrophysiol,
2012
Jan
, 35 (3-16).
514
Puppala D
et al.
Comparative gene expression profiling in human-induced pluripotent stem cell--derived cardiocytes and human and cynomolgus heart tissue.
Toxicol. Sci.,
2012
Jan
, 131 (292-301).
515
Kauferstein S
et al.
Cardiac channelopathy causing sudden death as revealed by molecular autopsy.
,
2012
Feb
28
, ().
516
Aidery P
et al.
Identification and functional characterization of the novel human ether-a-go-go-related gene (hERG) R744P mutant associated with hereditary long QT syndrome 2.
Biochem. Biophys. Res. Commun.,
2012
Feb
24
, 418 (830-5).
517
Yang X
et al.
Oestrogen upregulates L-type Ca²⁺ channels via oestrogen-receptor- by a regional genomic mechanism in female rabbit hearts.
J. Physiol. (Lond.),
2012
Feb
1
, 590 (493-508).
518
Lang F
et al.
Serum- and glucocorticoid-inducible kinase 1 in the regulation of renal and extrarenal potassium transport.
Clin. Exp. Nephrol.,
2012
Feb
, 16 (73-80).
519
Dennis AT
et al.
Molecular determinants of pentamidine-induced hERG trafficking inhibition.
Mol. Pharmacol.,
2012
Feb
, 81 (198-209).
520
Mayo S
et al.
Hypomethylation of the KCNQ1OT1 imprinting center of chromosome 11 associated to Sotos-like features.
J. Hum. Genet.,
2012
Feb
, 57 (153-6).
521
Chan PJ
et al.
Characterization of KCNQ1 atrial fibrillation mutations reveals distinct dependence on KCNE1.
J. Gen. Physiol.,
2012
Feb
, 139 (135-44).
522
O'Hara T
et al.
Arrhythmia formation in subclinical ("silent") long QT syndrome requires multiple insults: quantitative mechanistic study using the KCNQ1 mutation Q357R as example.
Heart Rhythm,
2012
Feb
, 9 (275-82).
523
Pareja K
et al.
Role of the activation gate in determining the extracellular potassium dependency of block of HERG by trapped drugs.
Channels (Austin),
2012
Dec
6
, 7 ().
524
Abriel H
et al.
Cardiac channelopathies: Genetic and molecular mechanisms.
Gene,
2012
Dec
22
, ().
525
Ishii K
et al.
Endocytic regulation of voltage-dependent potassium channels in the heart.
J. Pharmacol. Sci.,
2012
Dec
18
, 120 (264-9).
526
Friedrichs S
et al.
Modeling long QT syndromes using induced pluripotent stem cells: Current progress and future challenges.
Trends Cardiovasc. Med.,
2012
Dec
18
, ().
527
Organ-Darling LE
et al.
Interactions between hERG and KCNQ1 α-subunits are mediated by their C-termini and modulated by cAMP.
Am. J. Physiol. Heart Circ. Physiol.,
2012
Dec
15
, ().
528
Aidery P
et al.
Impaired ion channel function related to a common KCNQ1 mutation - implications for risk stratification in long QT syndrome 1.
Gene,
2012
Dec
10
, 511 (26-33).
529
Gaunt TR
et al.
Integration of Genetics into a Systems Model of Electrocardiographic Traits Using HumanCVD BeadChip.
Circ Cardiovasc Genet,
2012
Dec
1
, 5 (630-8).
530
Long J
et al.
Evaluation of genome-wide association study-identified type 2 diabetes loci in African Americans.
Am. J. Epidemiol.,
2012
Dec
1
, 176 (995-1001).
531
Wang Y
et al.
Probing the structural basis for differential KCNQ1 modulation by KCNE1 and KCNE2.
J. Gen. Physiol.,
2012
Dec
, 140 (653-69).
532
Turki A
et al.
Lack of association between genetic polymorphisms within KCNQ1 locus and type 2 diabetes in Tunisian Arabs.
Diabetes Res. Clin. Pract.,
2012
Dec
, 98 (452-8).
533
Winbo A
et al.
Prevalence, mutation spectrum, and cardiac phenotype of the Jervell and Lange-Nielsen syndrome in Sweden.
Europace,
2012
Dec
, 14 (1799-806).
534
Gamboa-Meléndez MA
et al.
Contribution of common genetic variation to the risk of type 2 diabetes in the Mexican Mestizo population.
Diabetes,
2012
Dec
, 61 (3314-21).
535
Fedoriw AM
et al.
Differentiation-driven nucleolar association of the mouse imprinted Kcnq1 locus.
G3 (Bethesda),
2012
Dec
, 2 (1521-8).
536
Olesen MS
et al.
High prevalence of long QT syndrome-associated SCN5A variants in patients with early-onset lone atrial fibrillation.
Circ Cardiovasc Genet,
2012
Aug
1
, 5 (450-9).
537
Song W
et al.
Cardiac sodium channel Nav1.5 mutations and cardiac arrhythmia.
Pediatr Cardiol,
2012
Aug
, 33 (943-9).
538
Wang M
et al.
Stoichiometry of the slow I(ks) potassium channel in human embryonic stem cell-derived myocytes.
Pediatr Cardiol,
2012
Aug
, 33 (938-42).
539
Iwata M
et al.
Genetic risk score constructed using 14 susceptibility alleles for type 2 diabetes is associated with the early onset of diabetes and may predict the future requirement of insulin injections among Japanese individuals.
Diabetes Care,
2012
Aug
, 35 (1763-70).
540
Mohammad F
et al.
Long noncoding RNA-mediated maintenance of DNA methylation and transcriptional gene silencing.
Development,
2012
Aug
, 139 (2792-803).
541
Ware JS
et al.
Paralogous annotation of disease-causing variants in long QT syndrome genes.
Hum. Mutat.,
2012
Aug
, 33 (1188-91).
542
Purtell K
et al.
The KCNQ1-KCNE2 K⁺ channel is required for adequate thyroid I⁻ uptake.
FASEB J.,
2012
Aug
, 26 (3252-9).
543
Chinushi M
et al.
Exercise-related QT interval shortening with a peaked T wave in a healthy boy with a family history of sudden cardiac death.
Pacing Clin Electrophysiol,
2012
Aug
, 35 (e239-42).
544
Jiménez-Vargas JM
et al.
Toxin modulators and blockers of hERG K(+) channels.
,
2012
Apr
5
, ().
545
Liu X
et al.
Characterization of A-935142, a hERG enhancer, in the presence and absence of standard hERG blockers.
Life Sci.,
2012
Apr
20
, 90 (607-11).
546
Zamorano-León JJ
et al.
KCNH2 Gene Mutation: A Potential Link Between Epilepsy and Long QT-2 Syndrome.
,
2012
Apr
19
, ().
547
Abd Allah ES
et al.
Postnatal development of transmural gradients in expression of ion channels and Ca(2+)-handling proteins in the ventricle.
,
2012
Apr
17
, ().
548
Vigneault P
et al.
Galantamine (Reminyl) delays cardiac ventricular repolarization and prolongs the QT interval by blocking the HERG current.
Eur. J. Pharmacol.,
2012
Apr
15
, 681 (68-74).
549
Park SE
et al.
Impact of common type 2 diabetes risk gene variants on future type 2 diabetes in the non-diabetic population in Korea.
J. Hum. Genet.,
2012
Apr
, 57 (265-8).
550
Chadha PS
et al.
Reduced KCNQ4-encoded voltage-dependent potassium channel activity underlies impaired β-adrenoceptor-mediated relaxation of renal arteries in hypertension.
Hypertension,
2012
Apr
, 59 (877-84).
551
Murphy LL
et al.
Developmentally regulated SCN5A splice variant potentiates dysfunction of a novel mutation associated with severe fetal arrhythmia.
Heart Rhythm,
2012
Apr
, 9 (590-7).
552
Couderc JP
et al.
Genotype- and Sex-Specific QT-RR Relationship in the Type-1 Long-QT Syndrome.
J Am Heart Assoc,
2012
Apr
, 1 (e000570).
553
Komarlu R
et al.
Fetal and neonatal presentation of long QT syndrome.
Pacing Clin Electrophysiol,
2012
Apr
, 35 (e87-90).
554
Lazarczyk MJ
et al.
Selective acquired long QT syndrome (saLQTS) upon risperidone treatment.
BMC Psychiatry,
2012
, 12 (220).
555
Xu XH
et al.
Allitridi inhibits multiple cardiac potassium channels expressed in HEK 293 cells.
PLoS ONE,
2012
, 7 (e51550).
556
Knoche JW
et al.
Atrial Fibrillation and Long QT Syndrome Presenting in a 12-Year-Old Girl.
Case Rep Pediatr,
2012
, 2012 (124838).
557
Lu S
et al.
Genome-wide association studies-derived susceptibility loci in type 2 diabetes: confirmation in a Chinese population.
Clin Invest Med,
2012
, 35 (E327).
558
Hoekstra M
et al.
Induced pluripotent stem cell derived cardiomyocytes as models for cardiac arrhythmias.
Front Physiol,
2012
, 3 (346).
559
Stump MR
et al.
Isoform-specific dominant-negative effects associated with hERG1 G628S mutation in long QT syndrome.
PLoS ONE,
2012
, 7 (e42552).
560
Krueger C
et al.
Pairing of homologous regions in the mouse genome is associated with transcription but not imprinting status.
PLoS ONE,
2012
, 7 (e38983).
561
Henrion U
et al.
Overlapping cardiac phenotype associated with a familial mutation in the voltage sensor of the KCNQ1 channel.
Cell. Physiol. Biochem.,
2012
, 29 (809-18).
562
Chen ZY
et al.
Structural and functional diversity of acidic scorpion potassium channel toxins.
PLoS ONE,
2012
, 7 (e35154).
563
Chen XD
et al.
Several polymorphisms of KCNQ1 gene are associated with plasma lipid levels in general Chinese populations.
PLoS ONE,
2012
, 7 (e34229).
564
Sato A
et al.
Long QT syndrome with nocturnal cardiac events caused by a KCNH2 missense mutation (G604S).
Intern. Med.,
2012
, 51 (1857-60).
565
Wang Y
et al.
Trafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum.
PLoS ONE,
2012
, 7 (e29885).
566
Choveau FS
et al.
Opposite Effects of the S4-S5 Linker and PIP(2) on Voltage-Gated Channel Function: KCNQ1/KCNE1 and Other Channels.
,
2012
, 3 (125).
567
van Vliet-Ostaptchouk JV
et al.
Common variants in the type 2 diabetes KCNQ1 gene are associated with impairments in insulin secretion during hyperglycaemic glucose clamp.
PLoS ONE,
2012
, 7 (e32148).
568
Guo X
et al.
IKs protects from ventricular arrhythmia during cardiac ischemia and reperfusion in rabbits by preserving the repolarization reserve.
PLoS ONE,
2012
, 7 (e31545).
569
Olesen MS
et al.
Mutations in the potassium channel subunit KCNE1 are associated with early-onset familial atrial fibrillation.
BMC Med. Genet.,
2012
, 13 (24).
570
Yang XF
et al.
The antibody targeting the e314 Peptide of human kv1.3 pore region serves as a novel, potent and specific channel blocker.
PLoS ONE,
2012
, 7 (e36379).
571
Morris LM
et al.
Mouse middle ear ion homeostasis channels and intercellular junctions.
PLoS ONE,
2012
, 7 (e39004).
572
Liu Z
et al.
Update on the slow delayed rectifier potassium current (I(Ks)): role in modulating cardiac function.
Curr. Med. Chem.,
2012
, 19 (1405-20).
573
Harley CA
et al.
Changes in channel trafficking and protein stability caused by LQT2 mutations in the PAS domain of the HERG channel.
PLoS ONE,
2012
, 7 (e32654).
574
Ferro F
et al.
Long-chain acylcarnitines regulate the hERG channel.
PLoS ONE,
2012
, 7 (e41686).
575
Moric-Janiszewska E
et al.
Molecular diagnostics of families with long-QT syndrome.
Cardiol J,
2012
, 19 (159-67).
576
Robbins KM
et al.
Expression of KCNQ1OT1, CDKN1C, H19, and PLAGL1 and the methylation patterns at the KvDMR1 and H19/IGF2 imprinting control regions is conserved between human and bovine.
J. Biomed. Sci.,
2012
, 19 (95).
577
Sun Q
et al.
The association between KCNQ1 gene polymorphism and type 2 diabetes risk: a meta-analysis.
PLoS ONE,
2012
, 7 (e48578).
578
Adalsteinsson BT
et al.
Heterogeneity in white blood cells has potential to confound DNA methylation measurements.
PLoS ONE,
2012
, 7 (e46705).
579
Zheng Y
et al.
Hexachlorophene is a potent KCNQ1/KCNE1 potassium channel activator which rescues LQTs mutants.
PLoS ONE,
2012
, 7 (e51820).
580
Sato A
et al.
Benign premature ventricular complexes from the right ventricular outflow tract triggered polymorphic ventricular tachycardia in a latent type 2 LQTS patient.
Intern. Med.,
2012
, 51 (3261-5).
581
Kansara B
et al.
Placement of an implantable cardioverter-defibrillator in an infant with congenital long QT syndrome: Anesthetic considerations.
Ann Card Anaesth,
2011 May-Aug
, 14 (122-6).
582
Patoine D
et al.
A novel KCNQ1 variant (L203P) associated with torsades de pointes-related syncope in a Steinert syndrome patient.
Can J Cardiol,
2011 Mar-Apr
, 27 (263.e5-12).
583
Dennis AT
et al.
Antidepressant-induced ubiquitination and degradation of the cardiac potassium channel hERG.
J. Biol. Chem.,
2011
Sep
30
, 286 (34413-25).
584
Malan D
et al.
Cardiomyocytes obtained from induced pluripotent stem cells with long-QT syndrome 3 recapitulate typical disease-specific features in vitro.
Circ. Res.,
2011
Sep
30
, 109 (841-7).
585
Nakajo K
et al.
Nano-environmental changes by KCNE proteins modify KCNQ channel function.
,
2011
Sep
1
, 5 ().
586
Marangoni S
et al.
A Brugada syndrome mutation (p.S216L) and its modulation by p.H558R polymorphism: standard and dynamic characterization.
Cardiovasc. Res.,
2011
Sep
1
, 91 (606-16).
587
Liu CT
et al.
Genetic association for renal traits among participants of African ancestry reveals new loci for renal function.
PLoS Genet.,
2011
Sep
, 7 (e1002264).
588
Mani BK
et al.
Activation of vascular KCNQ (Kv7) potassium channels reverses spasmogen-induced constrictor responses in rat basilar artery.
Br. J. Pharmacol.,
2011
Sep
, 164 (237-49).
589
Digby GC
et al.
Acquired long QT interval: a case series of multifactorial QT prolongation.
Clin Cardiol,
2011
Sep
, 34 (577-82).
590
Mistry HD
et al.
Novel expression and regulation of voltage-dependent potassium channels in placentas from women with preeclampsia.
Hypertension,
2011
Sep
, 58 (497-504).
591
Golding MC
et al.
Depletion of Kcnq1ot1 non-coding RNA does not affect imprinting maintenance in stem cells.
Development,
2011
Sep
, 138 (3667-78).
592
Guo J
et al.
Interaction between the cardiac rapidly (IKr) and slowly (IKs) activating delayed rectifier potassium channels revealed by low K+-induced hERG endocytic degradation.
J. Biol. Chem.,
2011
Oct
7
, 286 (34664-74).
593
Cheng J
et al.
LQTS-associated mutation A257G in α1-syntrophin interacts with the intragenic variant P74L to modify its biophysical phenotype.
Cardiogenetics,
2011
Oct
25
, 1 ().
594
Wang K
et al.
Biophysical properties of slow potassium channels in human embryonic stem cell derived cardiomyocytes implicate subunit stoichiometry.
,
2011
Oct
24
, ().
595
Saba S
et al.
Cardiac Levels of NOS1AP RNA from Right Ventricular Tissue Recovered During Lead Extraction.
,
2011
Oct
18
, ().
596
Schwoerer AP
et al.
Bupivacaine Destabilizes Action Potential Duration in Cellular and Computational Models of Long QT Syndrome 1.
,
2011
Oct
14
, ().
597
Palmer BR
et al.
KCNE5 Polymorphism rs697829 is Associated with QT Interval and Survival in Acute Coronary Syndromes Patients.
,
2011
Oct
10
, ().
598
Lemoine MD
et al.
Arrhythmogenic left atrial cellular electrophysiology in a murine genetic long QT syndrome model.
Cardiovasc. Res.,
2011
Oct
1
, 92 (67-74).
599
Barsheshet A
et al.
Risk of syncope in family members who are genotype-negative for a family-associated long-QT syndrome mutation.
Circ Cardiovasc Genet,
2011
Oct
, 4 (491-9).
600
Kanda VA
et al.
Protein kinase C downregulates I(Ks) by stimulating KCNQ1-KCNE1 potassium channel endocytosis.
Heart Rhythm,
2011
Oct
, 8 (1641-7).
601
Migdalovich D
et al.
Mutation and gender-specific risk in type 2 long QT syndrome: implications for risk stratification for life-threatening cardiac events in patients with long QT syndrome.
Heart Rhythm,
2011
Oct
, 8 (1537-43).
602
Urashima T
et al.
Enhancing effects of salicylate on quinidine-induced block of human wild type and LQT3 related mutant cardiac Na+ channels.
Biomed. Res.,
2011
Oct
, 32 (303-12).
603
Nof E
et al.
LQT5 masquerading as LQT2: a dominant negative effect of KCNE1-D85N rare polymorphism on KCNH2 current.
Europace,
2011
Oct
, 13 (1478-83).
604
Garg V
et al.
Molecular determinants for activation of human ether-à-go-go-related gene 1 potassium channels by 3-nitro-n-(4-phenoxyphenyl) benzamide.
Mol. Pharmacol.,
2011
Oct
, 80 (630-7).
605
Bonaldi A
et al.
Microduplication of the ICR2 domain at chromosome 11p15 and familial Silver-Russell syndrome.
Am. J. Med. Genet. A,
2011
Oct
, 155A (2479-83).
606
Tester DJ
et al.
Unexplained drownings and the cardiac channelopathies: a molecular autopsy series.
Mayo Clin. Proc.,
2011
Oct
, 86 (941-7).
607
Calloe K
et al.
Multiple arrhythmic syndromes in a newborn, owing to a novel mutation in SCN5A.
Can. J. Physiol. Pharmacol.,
2011
Oct
, 89 (723-36).
608
Kilinc OU
et al.
Successful Elimination of Significant Arrhythmia Burden with Flecainide in an Adolescent with Long QT Syndrome Type 3.
,
2011
Nov
30
, ().
609
Lahti AL
et al.
Human disease model for long QT syndrome type 2 using iPS cells demonstrates arrhythmogenic characteristics in cell culture.
,
2011
Nov
3
, ().
610
Aoki Y
et al.
Role of ion channels in sepsis-induced atrial tachyarrhythmias in guinea pigs.
,
2011
Nov
3
, ().
611
Kääb S
et al.
A Large Candidate Gene Survey Identifies the KCNE1 D85N Polymorphism as a Possible Modulator of Drug-Induced Torsades de Pointes.
,
2011
Nov
18
, ().
612
Alzamora R
et al.
Sexual dimorphism and oestrogen regulation of KCNE3 expression modulates the functional properties of KCNQ1 K⁺ channels.
J. Physiol. (Lond.),
2011
Nov
1
, 589 (5091-107).
613
Zhang X
et al.
KCNQ5/K(v)7.5 potassium channel expression and subcellular localization in primate retinal pigment epithelium and neural retina.
Am. J. Physiol., Cell Physiol.,
2011
Nov
, 301 (C1017-26).
614
Bai YL
et al.
HIV Tat protein inhibits hERG K+ channels: a potential mechanism of HIV infection induced LQTs.
J. Mol. Cell. Cardiol.,
2011
Nov
, 51 (876-80).
615
Saif-Ali R
et al.
KCNQ1 variants associate with type 2 diabetes in Malaysian Malay subjects.
Ann. Acad. Med. Singap.,
2011
Nov
, 40 (488-92).
616
Parvez B
et al.
The "missing" link in atrial fibrillation heritability.
J Electrocardiol,
2011
Nov
, 44 (641-4).
617
Kaufman ES
Arrhythmic risk in congenital long QT syndrome.
J Electrocardiol,
2011
Nov
, 44 (645-9).
618
Tsuji-Wakisaka K
et al.
Identification and functional characterization of KCNQ1 mutations around the exon 7-intron 7 junction affecting the splicing process.
Biochim. Biophys. Acta,
2011
Nov
, 1812 (1452-9).
619
Nakajo K
et al.
KCNQ1 subdomains involved in KCNE modulation revealed by an invertebrate KCNQ1 orthologue.
J. Gen. Physiol.,
2011
Nov
, 138 (521-35).
620
Lobarinas E
et al.
Effects of the potassium ion channel modulators BMS-204352 Maxipost and its R-enantiomer on salicylate-induced tinnitus in rats.
,
2011
May
27
, ().
621
Li Y
et al.
KCNE1 enhances phosphatidylinositol 4,5-bisphosphate (PIP2) sensitivity of IKs to modulate channel activity.
,
2011
May
16
, ().
622
Du X
et al.
Characteristics and molecular basis of celecoxib modulation on Kv7 potassium channels.
,
2011
May
13
, ().
623
Yanni J
et al.
Changes in Ion Channel Gene Expression Underlying Heart Failure-induced Sinoatrial Node Dysfunction.
,
2011
May
12
, ().
624
Ando F
et al.
Synergic effects of β-estradiol and erythromycin on hERG currents.
J. Membr. Biol.,
2011
May
, 241 (31-8).
625
Tavira B
et al.
KCNQ1 gene variants and risk of new-onset diabetes in tacrolimus-treated renal-transplanted patients.
Clin Transplant,
2011
May
, 25 (E284-91).
626
Horne AJ
et al.
A novel mechanism for LQT3 with 2:1 block: a pore-lining mutation in Nav1.5 significantly affects voltage-dependence of activation.
Heart Rhythm,
2011
May
, 8 (770-7).
627
Soma K
et al.
Abundant expression of KCNE1 in the left ventricle of the miniature pig.
Heart Vessels,
2011
May
, 26 (353-6).
628
Al-Khtib M
et al.
Vitrification at the germinal vesicle stage does not affect the methylation profile of H19 and KCNQ1OT1 imprinting centers in human oocytes subsequently matured in vitro.
Fertil. Steril.,
2011
May
, 95 (1955-60).
629
Guo J
et al.
Involvement of caveolin in probucol-induced reduction in hERG plasma-membrane expression.
Mol. Pharmacol.,
2011
May
, 79 (806-13).
630
Haapalahti P
et al.
Comparison of QT peak and QT end interval responses to autonomic adaptation in asymptomatic LQT1 mutation carriers.
Clin Physiol Funct Imaging,
2011
May
, 31 (209-14).
631
Nielsen T
et al.
Type 2 diabetes risk allele near CENTD2 is associated with decreased glucose-stimulated insulin release.
Diabetologia,
2011
May
, 54 (1052-6).
632
Jespersen T
Regulation and physiological function of Na(v) 1.5 and KCNQ1 channels.
Acta Physiol (Oxf),
2011
May
, 202 Suppl 683 (1-26).
633
Schwartz PJ
Pharmacological and Non-Pharmacological Management of the Congenital Long QT Syndrome: The Rationale.
,
2011
Mar
9
, ().
634
Ouillé A
et al.
ION CHANNEL BLOCKING PROFILE OF COMPOUNDS WITH REPORTED TORSADOGENIC EFFECTS: WHAT CAN BE LEARNED?
,
2011
Mar
8
, ().
635
Jons C
et al.
Use of Mutant-Specific Ion Channel Characteristics for Risk Stratification of Long QT Syndrome Patients.
Sci Transl Med,
2011
Mar
30
, 3 (76ra28).
636
Zhang T
et al.
LQTS mutation N1325S in cardiac sodium channel gene SCN5A causes cardiomyocyte apoptosis, cardiac fibrosis and contractile dysfunction in mice.
Int. J. Cardiol.,
2011
Mar
3
, 147 (239-45).
637
Wu D
et al.
Traditional Chinese formula, lubricating gut pill, stimulates cAMP-dependent CI(−) secretion across rat distal colonic mucosa.
J Ethnopharmacol,
2011
Mar
24
, 134 (406-13).
638
Song P
et al.
Kir4.1 channel expression is essential for parietal cell control of acid secretion.
,
2011
Mar
2
, ().
639
Matsa E
et al.
Drug evaluation in cardiomyocytes derived from human induced pluripotent stem cells carrying a long QT syndrome type 2 mutation.
,
2011
Mar
2
, ().
640
Novotny T
et al.
Mutation Analysis Ion Channel Genes Ventricular Fibrillation Survivors with Coronary Artery Disease.
,
2011
Mar
16
, ().
641
Yazawa M
et al.
Using induced pluripotent stem cells to investigate cardiac phenotypes in Timothy syndrome.
Nature,
2011
Mar
10
, 471 (230-4).
642
Papatheodoropoulos C
et al.
α5GABAA receptors regulate hippocampal sharp wave-ripple activity in vitro.
Neuropharmacology,
2011
Mar
, 60 (662-73).
643
Yu W
et al.
Effects of KCNQ1 polymorphisms on the therapeutic efficacy of oral antidiabetic drugs in Chinese patients with type 2 diabetes.
Clin. Pharmacol. Ther.,
2011
Mar
, 89 (437-42).
644
Skinner JR
et al.
Prospective, population-based long QT molecular autopsy study of postmortem negative sudden death in 1 to 40 year olds.
Heart Rhythm,
2011
Mar
, 8 (412-9).
645
Amorós I
et al.
Functional effects of a missense mutation in HERG associated with type 2 long QT syndrome.
Heart Rhythm,
2011
Mar
, 8 (463-70).
646
Kim Y
et al.
Suppression of CFTR-mediated Cl secretion of airway epithelium in vitamin C-deficient mice.
J. Korean Med. Sci.,
2011
Mar
, 26 (317-24).
647
Vangipurapu J
et al.
Association of indices of liver and adipocyte insulin resistance with 19 confirmed susceptibility loci for type 2 diabetes in 6,733 non-diabetic Finnish men.
Diabetologia,
2011
Mar
, 54 (563-71).
648
Gianulis EC
et al.
Rescue of aberrant gating by a genetically encoded PAS (Per-Arnt-Sim) domain in several long QT syndrome mutant human ether-á-go-go-related gene potassium channels.
J. Biol. Chem.,
2011
Jun
24
, 286 (22160-9).
649
Cheng EP
et al.
Restoration of Normal L-Type Ca2+ Channel Function During Timothy Syndrome by Ablation of an Anchoring Protein.
,
2011
Jun
23
, ().
650
Buber J
et al.
Risk of recurrent cardiac events after onset of menopause in women with congenital long-QT syndrome types 1 and 2.
Circulation,
2011
Jun
21
, 123 (2784-91).
651
Sroubek J
et al.
Protein kinase A activity at the endoplasmic reticulum surface is responsible for augmentation of human ether-a-go-go-related gene product (HERG).
J. Biol. Chem.,
2011
Jun
17
, 286 (21927-36).
652
Park KS
et al.
Identification and functional characterization of ion channels in CD34(+) hematopoietic stem cells from human peripheral blood.
,
2011
Jun
1
, ().
653
Mo J
et al.
Altered gene expression by low-dose arsenic exposure in humans and cultured cardiomyocytes: assessment by real-time PCR arrays.
Int J Environ Res Public Health,
2011
Jun
, 8 (2090-108).
654
Kanduri C
Kcnq1ot1: a chromatin regulatory RNA.
Semin. Cell Dev. Biol.,
2011
Jun
, 22 (343-50).
655
Vigneault P
et al.
Prolongation of cardiac ventricular repolarization under paliperidone: how and how much?
J. Cardiovasc. Pharmacol.,
2011
Jun
, 57 (690-5).
656
Rees SD
et al.
Replication of 13 genome-wide association (GWA)-validated risk variants for type 2 diabetes in Pakistani populations.
Diabetologia,
2011
Jun
, 54 (1368-74).
657
Rodriguez BA
et al.
Estrogen-mediated epigenetic repression of the imprinted gene cyclin-dependent kinase inhibitor 1C in breast cancer cells.
Carcinogenesis,
2011
Jun
, 32 (812-21).
658
Zhou PZ
et al.
Activation of human ether-a-go-go related gene (hERG) potassium channels by small molecules.
Acta Pharmacol. Sin.,
2011
Jun
, 32 (781-8).
659
Obata Y
et al.
Epigenetically immature oocytes lead to loss of imprinting during embryogenesis.
J. Reprod. Dev.,
2011
Jun
, 57 (327-34).
660
Neal AM
et al.
Renal defects in KCNE1 knockout mice are mimicked by chromanol 293B in vivo: identification of a KCNE1-regulated K+ conductance in the proximal tubule.
J. Physiol. (Lond.),
2011
Jul
15
, 589 (3595-609).
661
Tveito A
et al.
Defining candidate drug characteristics for Long-QT (LQT3) syndrome.
Math Biosci Eng,
2011
Jul
, 8 (861-73).
662
Allegue C
et al.
Prevalence of HCM and long QT syndrome mutations in young sudden cardiac death-related cases.
Int. J. Legal Med.,
2011
Jul
, 125 (565-72).
663
Jones MJ
et al.
An extended domain of Kcnq1ot1 silencing revealed by an imprinted fluorescent reporter.
Mol. Cell. Biol.,
2011
Jul
, 31 (2827-37).
664
Schroner Z
et al.
Variation in KCNQ1 is associated with therapeutic response to sulphonylureas.
Med. Sci. Monit.,
2011
Jul
, 17 (CR392-6).
665
Hassinen M
et al.
Thermal adaptation of the crucian carp (Carassius carassius) cardiac delayed rectifier current, IKs, by homomeric assembly of Kv7.1 subunits without MinK.
Am. J. Physiol. Regul. Integr. Comp. Physiol.,
2011
Jul
, 301 (R255-65).
666
Smith JL
et al.
Trafficking-deficient hERG K⁺ channels linked to long QT syndrome are regulated by a microtubule-dependent quality control compartment in the ER.
Am. J. Physiol., Cell Physiol.,
2011
Jul
, 301 (C75-85).
667
Couderc JP
et al.
T-wave morphology abnormalities in benign, potent, and arrhythmogenic I(kr) inhibition.
Heart Rhythm,
2011
Jul
, 8 (1036-43).
668
Wang RR
et al.
Novel compound heterozygous mutations T2C and 1149insT in the KCNQ1 gene cause Jervell and Lange-Nielsen syndrome.
Int. J. Mol. Med.,
2011
Jul
, 28 (41-6).
669
Li X
et al.
Bisoprolol reverses down-regulation of potassium channel proteins in ventricular tissues of rabbits with heart failure.
J Biomed Res,
2011
Jul
, 25 (274-279).
670
Choveau FS
et al.
KCNQ1 channels voltage dependence through a voltage-dependent binding of the S4-S5 linker to the pore domain.
J. Biol. Chem.,
2011
Jan
7
, 286 (707-16).
671
Labro AJ
et al.
The S4-S5 linker of KCNQ1 channels forms a structural scaffold with the S6 segment controlling gate closure.
J. Biol. Chem.,
2011
Jan
7
, 286 (717-25).
672
Barc J
et al.
Screening for copy number variation in genes associated with the long QT syndrome: clinical relevance.
J. Am. Coll. Cardiol.,
2011
Jan
4
, 57 (40-7).
673
Peal DS
et al.
Novel chemical suppressors of long QT syndrome identified by an in vivo functional screen.
Circulation,
2011
Jan
4
, 123 (23-30).
674
Abd Allah E
et al.
Changes in the expression of ion channels, connexins and Ca2+ handling proteins in the sinoatrial node during postnatal development.
,
2011
Jan
28
, ().
675
Thomas AM
et al.
Characterization of a binding site for anionic phospholipids on KCNQ1.
J. Biol. Chem.,
2011
Jan
21
, 286 (2088-100).
676
Kauferstein S
et al.
A novel mutation in the cardiac ryanodine receptor gene (RyR2) in a patient with an unequivocal LQTS.
Int. J. Cardiol.,
2011
Jan
21
, 146 (249-50).
677
Obiol-Pardo C
et al.
A Multiscale Simulation System for the Prediction of Drug-Induced Cardiotoxicity.
,
2011
Jan
20
, ().
678
Itzhaki I
et al.
Modelling the long QT syndrome with induced pluripotent stem cells.
,
2011
Jan
16
, ().
679
Aidery P
et al.
Biophysical properties of mutant KCNQ1 S277L channels linked to hereditary long QT syndrome with phenotypic variability.
,
2011
Jan
15
, ().
680
Millat G
et al.
Development of a high resolution melting method for the detection of genetic variations in Long QT Syndrome.
Clin. Chim. Acta,
2011
Jan
14
, 412 (203-7).
681
Han S
et al.
Fluconazole inhibits hERG K(+) channel by direct block and disruption of protein trafficking.
Eur. J. Pharmacol.,
2011
Jan
10
, 650 (138-44).
682
Stump MR
et al.
Multiple splicing defects caused by hERG splice site mutation 2592+1G>A associated with long QT syndrome.
Am. J. Physiol. Heart Circ. Physiol.,
2011
Jan
, 300 (H312-8).
683
Gong Q
et al.
Inhibition of nonsense-mediated mRNA decay by antisense morpholino oligonucleotides restores functional expression of hERG nonsense and frameshift mutations in long-QT syndrome.
J. Mol. Cell. Cardiol.,
2011
Jan
, 50 (223-9).
684
Huang H
et al.
Y1767C, a novel SCN5A mutation, induces a persistent Na+ current and potentiates ranolazine inhibition of Nav1.5 channels.
Am. J. Physiol. Heart Circ. Physiol.,
2011
Jan
, 300 (H288-99).
685
Parkman HP
et al.
Domperidone treatment for gastroparesis: demographic and pharmacogenetic characterization of clinical efficacy and side-effects.
Dig. Dis. Sci.,
2011
Jan
, 56 (115-24).
686
Viadero MT
et al.
Three generations of hereditary long-QT syndrome with complete penetrance caused by the p.G316E KCNQ1 mutation.
Pediatr Cardiol,
2011
Jan
, 32 (102-4).
687
Bartos DC
et al.
R231C mutation in KCNQ1 causes long QT syndrome type 1 and familial atrial fibrillation.
Heart Rhythm,
2011
Jan
, 8 (48-55).
688
Fröhlich H
et al.
Hypothyroidism of gene-targeted mice lacking Kcnq1.
Pflugers Arch.,
2011
Jan
, 461 (45-52).
689
Ernesto C
et al.
[Investigation of ion channel gene variants in patients with long QT syndrome.]
,
2011
Feb
4
, ().
690
Durdagi S
et al.
Combined receptor and ligand-based approach to the universal pharmacophore model development for studies of drug blockade to the hERG1 pore domain.
J Chem Inf Model,
2011
Feb
28
, 51 (463-74).
691
Nathan AT
et al.
Implications of Anesthesia in Children with Long QT Syndrome.
,
2011
Feb
23
, ().
692
Li P
et al.
Reciprocal control of hERG stability by Hsp70 and Hsc70 with implication for restoration of LQT2 mutant stability.
Circ. Res.,
2011
Feb
18
, 108 (458-68).
693
Ma LJ
et al.
Allosteric Features of KCNQ1 Gating Revealed by Alanine Scanning Mutagenesis.
Biophys. J.,
2011
Feb
16
, 100 (885-94).
694
Ciampa EJ
et al.
KCNE4 juxtamembrane region is required for interaction with calmodulin and for functional suppression of KCNQ1.
J. Biol. Chem.,
2011
Feb
11
, 286 (4141-9).
695
Dobríková M
et al.
[Relationship of the CDKAL1 and KCNQ1 gene polymorphisms to the age at diagnosis of type 2 diabetes in the Slovakian population].
Vnitr Lek,
2011
Feb
, 57 (155-8).
696
Alesutan I
et al.
Inhibition of the heterotetrameric K+ channel KCNQ1/KCNE1 by the AMP-activated protein kinase.
Mol. Membr. Biol.,
2011
Feb
, 28 (79-89).
697
Roepke TK
et al.
Genetic dissection reveals unexpected influence of beta subunits on KCNQ1 K+ channel polarized trafficking in vivo.
FASEB J.,
2011
Feb
, 25 (727-36).
698
Bentzen BH
et al.
Pharmacological activation of Kv11.1 in transgenic long QT-1 rabbits.
J. Cardiovasc. Pharmacol.,
2011
Feb
, 57 (223-30).
699
Wang YH
et al.
Gating-related molecular motions in the extracellular domain of the IKs channel: implications for IKs channelopathy.
J. Membr. Biol.,
2011
Feb
, 239 (137-56).
700
Li JL
et al.
[Association of age-related hearing loss with ion transporter KCNQ1 and NKCC1 in cochlea of C57BL/6J mice].
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi,
2011
Feb
, 46 (139-43).
701
Davies MR
et al.
An In silico Canine Cardiac Midmyocardial Action Potential Duration Model as a Tool for Early Drug Safety Assessment.
,
2011
Dec
23
, ().
702
703
Coey AT
et al.
Reconstitution of KCNE1 into lipid bilayers: comparing the structural, dynamic, and activity differences in micelle and vesicle environments.
Biochemistry,
2011
Dec
20
, 50 (10851-9).
704
Yamazaki K
et al.
A novel method of selecting human embryonic stem cell-derived cardiomyocyte clusters for assessment of potential to influence QT interval.
,
2011
Dec
14
, ().
705
706
Sung RK
et al.
QTc prolongation and family history of sudden death in a patient with desmin cardiomyopathy.
Pacing Clin Electrophysiol,
2011
Dec
, 34 (e105-8).
707
Krause U
et al.
A rare association of long QT syndrome and syndactyly: Timothy syndrome (LQT 8).
Clin Res Cardiol,
2011
Dec
, 100 (1123-7).
708
Chen J
et al.
A dual mechanism for I(Ks) current reduction by the pathogenic mutation KCNQ1-S277L.
Pacing Clin Electrophysiol,
2011
Dec
, 34 (1652-64).
709
Maillé E
et al.
Regulation of normal and cystic fibrosis airway epithelial repair processes by TNF-α after injury.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2011
Dec
, 301 (L945-55).
710
Mikuni I
et al.
Partial restoration of the long QT syndrome associated KCNQ1 A341V mutant by the KCNE1 β-subunit.
Biochim. Biophys. Acta,
2011
Dec
, 1810 (1285-93).
711
Trudeau MC
et al.
hERG1a N-terminal eag domain-containing polypeptides regulate homomeric hERG1b and heteromeric hERG1a/hERG1b channels: a possible mechanism for long QT syndrome.
J. Gen. Physiol.,
2011
Dec
, 138 (581-92).
712
Aziz PF
et al.
Genotype- and mutation site-specific QT adaptation during exercise, recovery, and postural changes in children with long-QT syndrome.
Circ Arrhythm Electrophysiol,
2011
Dec
, 4 (867-73).
713
Roepke TK
et al.
KCNE2 forms potassium channels with KCNA3 and KCNQ1 in the choroid plexus epithelium.
FASEB J.,
2011
Dec
, 25 (4264-73).
714
Shao C
et al.
Electrophysiological study of V535M hERG mutation of LQT2.
J. Huazhong Univ. Sci. Technol. Med. Sci.,
2011
Dec
, 31 (741-8).
715
Goldenberg I
et al.
Trigger-specific ion-channel mechanisms, risk factors, and response to therapy in type 1 long QT syndrome.
,
2011
Aug
24
, ().
716
Larsen AP
et al.
Extracellular potassium inhibits Kv7.1 potassium channels by stabilizing an inactivated state.
Biophys. J.,
2011
Aug
17
, 101 (818-27).
717
Alzamora R
et al.
Estrogen inhibits chloride secretion caused by cholera and Escherichia coli enterotoxins in female rat distal colon.
Steroids,
2011
Aug
, 76 (867-76).
718
Lin S
et al.
Nonallelic transcriptional roles of CTCF and cohesins at imprinted loci.
Mol. Cell. Biol.,
2011
Aug
, 31 (3094-104).
719
Fougere RR
et al.
Functional characterization of the LQT2-causing mutation R582C and the associated voltage-dependent fluorescence signal.
Heart Rhythm,
2011
Aug
, 8 (1273-80).
720
Zarraga IG
et al.
Nonsense-mediated mRNA decay caused by a frameshift mutation in a large kindred of type 2 long QT syndrome.
Heart Rhythm,
2011
Aug
, 8 (1200-6).
721
Takahara A
et al.
Measurements of Cardiac Ion Channel Subunits in the Chronic Atrioventricular Block Dog.
,
2011
Apr
21
, ().
722
Horigome H
et al.
Detection of Extra-Components of T Wave by Independent Component Analysis in Congenital Long QT Syndrome.
,
2011
Apr
21
, ().
723
Yamagata K
et al.
Voltage-gated K+ channel KCNQ1 regulates insulin secretion in MIN6 β-cell line.
Biochem. Biophys. Res. Commun.,
2011
Apr
15
, 407 (620-5).
724
Ohno S
et al.
KCNE5 (KCNE1L) Variants Are Novel Modulator of Brugada Syndrome and Idiopathic Ventricular Fibrillation.
,
2011
Apr
14
, ().
725
Albesa M
et al.
Nedd4-2 dependent ubiquitylation and regulation of the cardiac potassium channel hERG.
,
2011
Apr
1
, ().
726
El Gebeily G
et al.
Upregulation of ventricular potassium channels by chronic tamoxifen treatment.
Cardiovasc. Res.,
2011
Apr
1
, 90 (68-76).
727
Hekkala AM
et al.
Epinephrine bolus test in detecting long QT syndrome mutation carriers with indeterminable electrocardiographic phenotype.
Ann Noninvasive Electrocardiol,
2011
Apr
, 16 (172-9).
728
Grilo LS
et al.
Patient with syncope and LQTS carrying a mutation in the PAS domain of the hERG1 channel.
Ann Noninvasive Electrocardiol,
2011
Apr
, 16 (213-8).
729
Van Horn WD
et al.
Working model for the structural basis for KCNE1 modulation of the KCNQ1 potassium channel.
Curr. Opin. Struct. Biol.,
2011
Apr
, 21 (283-91).
730
Cooper EC
Made for "anchorin": Kv7.2/7.3 (KCNQ2/KCNQ3) channels and the modulation of neuronal excitability in vertebrate axons.
Semin. Cell Dev. Biol.,
2011
Apr
, 22 (185-92).
731
Winbo A
et al.
Origin of the Swedish long QT syndrome Y111C/KCNQ1 founder mutation.
Heart Rhythm,
2011
Apr
, 8 (541-7).
732
Andersen MN
et al.
Kv7.1 surface expression is regulated by epithelial cell polarization.
Am. J. Physiol., Cell Physiol.,
2011
Apr
, 300 (C814-24).
733
Rice KS
et al.
Elevated serum gastrin levels in Jervell and Lange-Nielsen syndrome: a marker of severe KCNQ1 dysfunction?
Heart Rhythm,
2011
Apr
, 8 (551-4).
734
Moric Janiszewska E
et al.
Expression of genes KCNQ1 and HERG encoding potassium ion channels Ik(r), Ik(s) in long QT syndrome.
Kardiol Pol,
2011
, 69 (423-9).
735
Alzamora R
et al.
Berberine Reduces cAMP-Induced Chloride Secretion in T84 Human Colonic Carcinoma Cells through Inhibition of Basolateral KCNQ1 Channels.
Front Physiol,
2011
, 2 (33).
736
Walzik S
et al.
Alternative splicing of the cardiac sodium channel creates multiple variants of mutant T1620K channels.
PLoS ONE,
2011
, 6 (e19188).
737
Frolov RV
et al.
Inhibition of HERG potassium channels by celecoxib and its mechanism.
PLoS ONE,
2011
, 6 (e26344).
738
Rotte A
et al.
Ca2+ activated K+ channel Kca3.1 as a determinant of gastric acid secretion.
Cell. Physiol. Biochem.,
2011
, 27 (597-604).
739
Strutz-Seebohm N
et al.
Structural basis of slow activation gating in the cardiac I Ks channel complex.
Cell. Physiol. Biochem.,
2011
, 27 (443-52).
741
Mihic A
et al.
Trafficking defect and proteasomal degradation contribute to the phenotype of a novel KCNH2 long QT syndrome mutation.
PLoS ONE,
2011
, 6 (e18273).
742
Liu X
et al.
ENU mutagenesis screen to establish motor phenotypes in wild-type mice and modifiers of a pre-existing motor phenotype in tau mutant mice.
J. Biomed. Biotechnol.,
2011
, 2011 (130947).
743
Algar E
et al.
An 11p15 imprinting centre region 2 deletion in a family with Beckwith Wiedemann syndrome provides insights into imprinting control at CDKN1C.
PLoS ONE,
2011
, 6 (e29034).
744
Cavaliere S
et al.
Drosophila KCNQ channel displays evolutionarily conserved electrophysiology and pharmacology with mammalian KCNQ channels.
PLoS ONE,
2011
, 6 (e23898).
745
Yun J
et al.
Hirsutenone directly blocks human ether-a-go-go related gene K+ channels.
Biol. Pharm. Bull.,
2011
, 34 (1815-22).
746
Girmatsion Z
et al.
N-terminal arginines modulate plasma-membrane localization of Kv7.1/KCNE1 channel complexes.
PLoS ONE,
2011
, 6 (e26967).
747
Saif-Ali R
et al.
KCNQ1 haplotypes associate with type 2 diabetes in Malaysian Chinese Subjects.
Int J Mol Sci,
2011
, 12 (5705-18).
748
Been LF
et al.
Variants in KCNQ1 increase type II diabetes susceptibility in South Asians: a study of 3,310 subjects from India and the US.
BMC Med. Genet.,
2011
, 12 (18).
749
Namekata I
et al.
Blocking effect of NIP-142 on the KCNQ1/KCNE1 channel current expressed in HEK293 cells.
Biol. Pharm. Bull.,
2011
, 34 (153-5).
750
Nekouzadeh A
et al.
Continuum Molecular Simulation of Large Conformational Changes during Ion-Channel Gating.
PLoS ONE,
2011
, 6 (e20186).
751
Lahtinen AM
et al.
KCNE1 D85N polymorphism - a sex-specific modifier in type 1 long QT syndrome?
BMC Med. Genet.,
2011
, 12 (11).
752
Couderc JP
et al.
Short and long QT syndromes: does QT length really matter?
J Electrocardiol,
2010 Sep-Oct
, 43 (396-9).
753
Lang F
et al.
SGK, renal function and hypertension.
J. Nephrol.,
2010 Nov-Dec
, 23 Suppl 16 (S124-9).
754
Cirović S
et al.
Differential expression of KCNQ1 K+ channel in tubular cells of frog kidney.
Eur J Histochem,
2010 Jan-Mar
, 54 (e7).
755
Shah DP
et al.
Ranolazine Safely Decreases Ventricular and Atrial Fibrillation in Timothy Syndrome (LQT8).
,
2010
Sep
30
, ().
756
Alzamora R
et al.
AMP-Activated Protein Kinase Inhibits KCNQ1 Channels Through Regulation of the Ubiquitin Ligase Nedd4-2 in Renal Epithelial Cells.
,
2010
Sep
22
, ().
757
Haugaa KH
et al.
Transmural Differences in Myocardial Contraction in Long-QT Syndrome. Mechanical Consequences of Ion Channel Dysfunction.
,
2010
Sep
20
, ().
758
Piron J
et al.
KCNE1-KCNQ1 osmoregulation by interaction of phosphatidylinositol-4,5-bisphosphate with Mg2+ and polyamines.
J. Physiol. (Lond.),
2010
Sep
15
, 588 (3471-83).
759
Ng FL
et al.
Expression and function of the K(+) channel KCNQ genes in human arteries.
,
2010
Sep
14
, ().
760
Ren XQ
et al.
Pore Mutants of HERG and KvLQT1 Downregulate the Reciprocal Currents in Stable Cell Lines.
,
2010
Sep
10
, ().
761
Zhong XZ
et al.
Participation of KCNQ (Kv7) potassium channels in myogenic control of cerebral arterial diameter.
J. Physiol. (Lond.),
2010
Sep
1
, 588 (3277-93).
762
Nanda Kumar NS
et al.
Mucosal potassium efflux mediated via Kcnn4 channels provides the driving force for electrogenic anion secretion in colon.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2010
Sep
, 299 (G707-14).
763
Bokil NJ
et al.
Molecular genetics of long QT syndrome.
Mol. Genet. Metab.,
2010
Sep
, 101 (1-8).
764
Moretti A
et al.
Patient-specific induced pluripotent stem-cell models for long-QT syndrome.
N. Engl. J. Med.,
2010
Oct
7
, 363 (1397-409).
765
Nakajo K
et al.
Stoichiometry of the KCNQ1 - KCNE1 ion channel complex.
,
2010
Oct
20
, ().
766
Tester DJ
et al.
Prevalence and spectrum of large deletions or duplications in the major long QT syndrome-susceptibility genes and implications for long QT syndrome genetic testing.
Am. J. Cardiol.,
2010
Oct
15
, 106 (1124-8).
767
Lian J
et al.
Novel characteristics of a trafficking-defective G572R-hERG channel linked to hereditary long QT syndrome.
Can J Cardiol,
2010
Oct
, 26 (417-22).
768
Kuryshev YA
et al.
Increased cardiac risk in concomitant methadone and diazepam treatment: pharmacodynamic interactions in cardiac ion channels.
J. Cardiovasc. Pharmacol.,
2010
Oct
, 56 (420-30).
769
Furushima H
et al.
Fetal atrioventricular block and postpartum augmentative QT prolongation in a patient with long-QT syndrome with KCNQ1 mutation.
J. Cardiovasc. Electrophysiol.,
2010
Oct
, 21 (1170-3).
770
Dautova Y
et al.
Atrial arrhythmogenic properties in wild-type and Scn5a+/- murine hearts.
Exp. Physiol.,
2010
Oct
, 95 (994-1007).
771
Baek JS
et al.
Jervell and Lange-Nielsen syndrome: novel compound heterozygous mutations in the KCNQ1 in a Korean family.
J. Korean Med. Sci.,
2010
Oct
, 25 (1522-5).
772
Hammoud SS
et al.
Alterations in sperm DNA methylation patterns at imprinted loci in two classes of infertility.
Fertil. Steril.,
2010
Oct
, 94 (1728-33).
773
Oka Y
et al.
Atrioventricular block-induced Torsades de Pointes with clinical and molecular backgrounds similar to congenital long QT syndrome.
Circ. J.,
2010
Nov
25
, 74 (2562-71).
774
Subbotina J
et al.
Structural refinement of the hERG1 pore and voltage-sensing domains with ROSETTA-membrane and molecular dynamics simulations.
Proteins,
2010
Nov
1
, 78 (2922-34).
775
Sy RW
et al.
Repolarization dynamics during exercise discriminate between LQT1 and LQT2 genotypes.
J. Cardiovasc. Electrophysiol.,
2010
Nov
, 21 (1242-6).
776
Purtell K
et al.
Cardiac arrhythmia and thyroid dysfunction: a novel genetic link.
Int. J. Biochem. Cell Biol.,
2010
Nov
, 42 (1767-70).
779
Roura-Ferrer M
et al.
Impact of KCNE subunits on KCNQ1 (Kv7.1) channel membrane surface targeting.
J. Cell. Physiol.,
2010
Nov
, 225 (692-700).
780
Park JH
et al.
High expression of large-conductance Ca(2+)-activated K(+) channel in the CD133(+) subpopulation of SH-SY5Y neuroblastoma cells.
,
2010
May
7
, ().
781
Kang J
et al.
In Vitro Electrocardiographic and Cardiac Ion Channel Effects of (-)-Epigallocatechin-3-Gallate, the Main Catechin of Green Tea.
,
2010
May
18
, ().
782
Matsuzaki J
et al.
Acid Suppression by Proton Pump Inhibitors Enhances Aquaporin-4 and KCNQ1 Expression in Gastric Fundic Parietal Cells in Mouse.
,
2010
May
1
, ().
783
Eldstrom J
et al.
Mechanistic basis for LQT1 caused by S3 mutations in the KCNQ1 subunit of IKs.
J. Gen. Physiol.,
2010
May
, 135 (433-48).
784
Allegue C
et al.
A new approach to long QT syndrome mutation detection by Sequenom MassARRAY system.
Electrophoresis,
2010
May
, 31 (1648-55).
785
Dong MQ
et al.
Regulation of human cardiac KCNQ1/KCNE1 channel by epidermal growth factor receptor kinase.
Biochim. Biophys. Acta,
2010
May
, 1798 (995-1001).
786
Svendsen JH
et al.
Screening for, and management of, possible arrhythmogenic syndromes (channelopathies/ion channel diseases).
Europace,
2010
May
, 12 (741-2).
787
Navedo MF
et al.
Increased coupled gating of L-type Ca2+ channels during hypertension and Timothy syndrome.
Circ. Res.,
2010
Mar
5
, 106 (748-56).
788
Preston P
et al.
Disruption of the K+ channel beta-subunit KCNE3 reveals an important role in intestinal and tracheal Cl- transport.
J. Biol. Chem.,
2010
Mar
5
, 285 (7165-75).
789
Zheng R
et al.
Analysis of the interactions between the C-terminal cytoplasmic domains of KCNQ1 and KCNE1 channel subunits.
,
2010
Mar
2
, ().
790
Leong IU
et al.
Zebrafish as a model for Long QT syndrome: the evidence, and the means of manipulating zebrafish gene expression.
,
2010
Mar
19
, ().
792
Pan Q
et al.
KCNQ1 loss-of-function mutation impairs gastric acid secretion in mice.
Mol. Biol. Rep.,
2010
Mar
, 37 (1329-33).
793
Summers KM
et al.
Mutations at KCNQ1 and an unknown locus cause long QT syndrome in a large Australian family: implications for genetic testing.
Am. J. Med. Genet. A,
2010
Mar
, 152A (613-21).
794
Gray C
et al.
Expression of a common LQT1 mutation in five apparently unrelated families in a regional inherited arrhythmia clinic.
J. Cardiovasc. Electrophysiol.,
2010
Mar
, 21 (296-300).
795
Amin AS
et al.
Fever-triggered ventricular arrhythmias in Brugada syndrome and type 2 long-QT syndrome.
Neth Heart J,
2010
Mar
, 18 (165-9).
796
Fink M
et al.
Pharmacodynamic effects in the cardiovascular system: the modeller's view.
Basic Clin. Pharmacol. Toxicol.,
2010
Mar
, 106 (243-9).
797
Vanoye CG
et al.
KCNQ1/KCNE1 assembly, co-translation not required.
Channels (Austin),
2010
Mar
, 4 (108-14).
798
Itoh H
et al.
Long QT Syndrome with Compound Mutations is Associated with a More Severe Phenotype :A Japanese Multicenter Study.
,
2010
Jun
8
, ().
799
Lang F
et al.
Significance of SGK1 in the regulation of neuronal function.
,
2010
Jun
7
, ().
800
Yang Y
et al.
Identification of a Kir3.4 mutation in congenital long QT syndrome.
Am. J. Hum. Genet.,
2010
Jun
11
, 86 (872-80).
801
Valdivia CR
et al.
Loss-of-function mutation of the SCN3B-encoded sodium channel {beta}3 subunit associated with a case of idiopathic ventricular fibrillation.
Cardiovasc. Res.,
2010
Jun
1
, 86 (392-400).
802
Lvov A
et al.
Identification of a protein-protein interaction between KCNE1 and the activation gate machinery of KCNQ1.
J. Gen. Physiol.,
2010
Jun
, 135 (607-18).
803
Zhou JB
et al.
Variants in KCNQ1, AP3S1, MAN2A1, and ALDH7A1 and the risk of type 2 diabetes in the Chinese Northern Han population: a case-control study and meta-analysis.
Med. Sci. Monit.,
2010
Jun
, 16 (BR179-83).
804
Lin EC
et al.
Properties of WT and mutant hERG K(+) channels expressed in neonatal mouse cardiomyocytes.
Am. J. Physiol. Heart Circ. Physiol.,
2010
Jun
, 298 (H1842-9).
805
Wu D
et al.
State-dependent electrostatic interactions of S4 arginines with E1 in S2 during Kv7.1 activation.
J. Gen. Physiol.,
2010
Jun
, 135 (595-606).
806
Labro AJ
et al.
The rate-dependent biophysical properties of the LQT1 H258R mutant are counteracted by a dominant negative effect on channel trafficking.
J. Mol. Cell. Cardiol.,
2010
Jun
, 48 (1096-104).
807
808
809
810
Choi SH
et al.
Ginsenoside Rg3 activates human KCNQ1 K+ channel currents through interacting with the K318 and V319 residues: a role of KCNE1 subunit.
Eur. J. Pharmacol.,
2010
Jul
10
, 637 (138-47).
811
Fabritz L
et al.
Autonomic modulation and antiarrhythmic therapy in a model of long QT syndrome type 3.
Cardiovasc. Res.,
2010
Jul
1
, 87 (60-72).
813
Voight BF
et al.
Twelve type 2 diabetes susceptibility loci identified through large-scale association analysis.
Nat. Genet.,
2010
Jul
, 42 (579-89).
814
Tester DJ
et al.
Epidemiologic, molecular, and functional evidence suggest A572D-SCN5A should not be considered an independent LQT3-susceptibility mutation.
Heart Rhythm,
2010
Jul
, 7 (912-9).
815
Hayashi K
et al.
Trafficking-competent KCNQ1 variably influences the function of HERG long QT alleles.
Heart Rhythm,
2010
Jul
, 7 (973-80).
816
817
Market-Velker BA
et al.
Dual effects of superovulation: loss of maternal and paternal imprinted methylation in a dose-dependent manner.
Hum. Mol. Genet.,
2010
Jan
1
, 19 (36-51).
818
Ponte ML
et al.
Mechanisms of Drug Induced QT Interval Prolongation.
Curr Drug Saf,
2010
Jan
1
, 5 (44-53).
819
Sung RJ
et al.
Beta-adrenergic modulation of arrhythmogenesis and identification of targeted sites of antiarrhythmic therapy in Timothy (LQT8) syndrome: a theoretical study.
Am. J. Physiol. Heart Circ. Physiol.,
2010
Jan
, 298 (H33-44).
820
Chen Z
et al.
Association study of four variants in KCNQ1 with type 2 diabetes mellitus and premature coronary artery disease in a Chinese population.
Mol. Biol. Rep.,
2010
Jan
, 37 (207-12).
821
Tremblay AM
et al.
Physiological genomics identifies estrogen-related receptor alpha as a regulator of renal sodium and potassium homeostasis and the renin-angiotensin pathway.
Mol. Endocrinol.,
2010
Jan
, 24 (22-32).
822
Shin HD
et al.
Association of KCNQ1 polymorphisms with the gestational diabetes mellitus in Korean women.
J. Clin. Endocrinol. Metab.,
2010
Jan
, 95 (445-9).
823
Lang F
et al.
Heterocyclic indazole derivatives as SGK1 inhibitors, WO2008138448.
Expert Opin Ther Pat,
2010
Jan
, 20 (129-35).
824
Matavel A
et al.
PKA and PKC partially rescue long QT type 1 phenotype by restoring channel-PIP2 interactions.
Channels (Austin),
2010
Jan
, 4 (3-11).
825
Alvarez PA
et al.
QT alterations in psychopharmacology: proven candidates and suspects.
Curr Drug Saf,
2010
Jan
, 5 (97-104).
826
Thomas D
et al.
Biophysical characterization of KCNQ1 P320 mutations linked to long QT syndrome 1.
J. Mol. Cell. Cardiol.,
2010
Jan
, 48 (230-7).
827
Abraham RL
et al.
Augmented potassium current is a shared phenotype for two genetic defects associated with familial atrial fibrillation.
J. Mol. Cell. Cardiol.,
2010
Jan
, 48 (181-90).
828
Charpentier F
et al.
Delayed rectifier K(+) currents and cardiac repolarization.
J. Mol. Cell. Cardiol.,
2010
Jan
, 48 (37-44).
829
Banderali U
et al.
Impaired stretch modulation in potentially lethal cardiac sodium channel mutants.
Channels (Austin),
2010
Jan
, 4 (12-21).
830
Mashanov GI
et al.
Direct observation of individual KCNQ1 potassium channels reveals their distinctive diffusive behavior.
J. Biol. Chem.,
2010
Feb
5
, 285 (3664-75).
831
Massaeli H
et al.
Extracellular K+ Is a Prerequisite for the Function and Plasma Membrane Stability of HERG Channels.
,
2010
Feb
4
, ().
832
Kang C
et al.
Functional delivery of a membrane protein into oocyte membranes using bicelles.
Biochemistry,
2010
Feb
2
, 49 (653-5).
833
Bal M
et al.
Ca2+/calmodulin disrupts AKAP79/150 interactions with KCNQ (M-Type) K+ channels.
J. Neurosci.,
2010
Feb
10
, 30 (2311-23).
834
Saba S
et al.
Effect of right ventricular versus biventricular pacing on electrical remodeling in the normal heart.
Circ Arrhythm Electrophysiol,
2010
Feb
1
, 3 (79-87).
835
Grunnet M
Repolarization of the cardiac action potential. Does an increase in repolarization capacity constitute a new anti-arrhythmic principle?
Acta Physiol (Oxf),
2010
Feb
, 198 Suppl 676 (1-48).
836
Harmer SC
et al.
Mechanisms of disease pathogenesis in long QT syndrome type 5.
Am. J. Physiol., Cell Physiol.,
2010
Feb
, 298 (C263-73).
837
Mansén A
et al.
Thyroid hormone receptor alpha can control action potential duration in mouse ventricular myocytes through the KCNE1 ion channel subunit.
Acta Physiol (Oxf),
2010
Feb
, 198 (133-42).
838
Jing H
et al.
Ephedrine controls heart rhythms by activating cardiac I(ks) currents.
J. Cardiovasc. Pharmacol.,
2010
Feb
, 55 (145-52).
839
Lian J
et al.
[A novel mutation of the KCNH2 gene in a family with congenital long QT syndrome]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2010
Feb
, 27 (77-80).
840
Grallert H
et al.
Association of genetic variation in KCNQ1 with type 2 diabetes in the KORA surveys.
Horm. Metab. Res.,
2010
Feb
, 42 (149-51).
841
Eggermann T
et al.
Identification of a 21q22 duplication in a Silver-Russell syndrome patient further narrows down the Down syndrome critical region.
Am. J. Med. Genet. A,
2010
Feb
, 152A (356-9).
842
Serratrice G
et al.
[Potassium channelopathies and Morvan's syndromes].
Bull. Acad. Natl. Med.,
2010
Feb
, 194 (391-406; discussion 406-7).
843
Osteen JD
et al.
KCNE1 alters the voltage sensor movements necessary to open the KCNQ1 channel gate.
Proc. Natl. Acad. Sci. U.S.A.,
2010
Dec
28
, 107 (22710-5).
844
Wu D
et al.
KCNE1 remodels the voltage sensor of Kv7.1 to modulate channel function.
Biophys. J.,
2010
Dec
1
, 99 (3599-608).
845
Cao X
et al.
Cardiac ion channel safety profiling on the IonWorks Quattro automated patch clamp system.
Assay Drug Dev Technol,
2010
Dec
, 8 (766-80).
846
Schimpf R
et al.
Arrhythmogenic hereditary syndromes: Brugada Syndrome, long QT syndrome, short QT syndrome and CPVT.
Minerva Cardioangiol,
2010
Dec
, 58 (623-36).
847
Soh H
et al.
The specific slow afterhyperpolarization inhibitor UCL2077 is a subtype-selective blocker of the epilepsy associated KCNQ channels.
Mol. Pharmacol.,
2010
Dec
, 78 (1088-95).
848
Kim HT
et al.
Long QT syndrome provoked by induction of general anesthesia -A case report-.
Korean J Anesthesiol,
2010
Dec
, 59 Suppl (S114-8).
849
Blana A
et al.
Knock-in gain-of-function sodium channel mutation prolongs atrial action potentials and alters atrial vulnerability.
Heart Rhythm,
2010
Dec
, 7 (1862-9).
850
Burashnikov E
et al.
Mutations in the cardiac L-type calcium channel associated with inherited J-wave syndromes and sudden cardiac death.
Heart Rhythm,
2010
Dec
, 7 (1872-82).
851
Kim JA
et al.
Trigger-specific risk factors and response to therapy in long QT syndrome type 2.
Heart Rhythm,
2010
Dec
, 7 (1797-805).
852
El Harchi A
et al.
The S140G KCNQ1 atrial fibrillation mutation affects 'I(KS)' profile during both atrial and ventricular action potentials.
J. Physiol. Pharmacol.,
2010
Dec
, 61 (759-64).
853
Macías A
et al.
Celecoxib blocks cardiac Kv1.5, Kv4.3 and Kv7.1 (KCNQ1) channels: effects on cardiac action potentials.
J. Mol. Cell. Cardiol.,
2010
Dec
, 49 (984-92).
854
Cordeiro JM
et al.
Overlapping LQT1 and LQT2 phenotype in a patient with long QT syndrome associated with loss-of-function variations in KCNQ1 and KCNH2.
Can. J. Physiol. Pharmacol.,
2010
Dec
, 88 (1181-90).
855
Hori N
et al.
Aberrant CpG methylation of the imprinting control region KvDMR1 detected in assisted reproductive technology-produced calves and pathogenesis of large offspring syndrome.
Anim. Reprod. Sci.,
2010
Dec
, 122 (303-12).
856
Rodriguez N
et al.
Phosphatidylinositol-4,5-Bisphosphate (PIP(2)) Stabilizes the Open Pore Conformation of the Kv11.1 (hERG) Channel.
,
2010
Aug
9
, 99 (1110-1118).
858
Durdagi S
et al.
Insights into the Molecular Mechanism of hERG1 Channel Activation and Blockade by Drugs.
,
2010
Aug
26
, ().
859
Tu E
et al.
Post-Mortem Review and Genetic Analysis of Sudden Unexpected Death in Epilepsy (SUDEP) Cases.
,
2010
Aug
24
, ().
860
Choufani S
et al.
Beckwith-Wiedemann syndrome.
Am J Med Genet C Semin Med Genet,
2010
Aug
15
, 154C (343-54).
861
Weaver JR
et al.
Domain-specific response of imprinted genes to reduced DNMT1.
Mol. Cell. Biol.,
2010
Aug
, 30 (3916-28).
862
Stead LF
et al.
KvDB; mining and mapping sequence variants in voltage-gated potassium channels.
Hum. Mutat.,
2010
Aug
, 31 (908-17).
863
Albert CM
et al.
Common Variants in Cardiac Ion Channel Genes Are Associated with Sudden Cardiac Death.
,
2010
Apr
17
, ().
864
Lee HA
et al.
Electrophysiological Effects of the Anti-Cancer Drug Lapatinib on Cardiac Repolarization.
,
2010
Apr
12
, ().
865
Wong JA
et al.
Utility of treadmill testing in identification and genotype prediction in long-QT syndrome.
Circ Arrhythm Electrophysiol,
2010
Apr
1
, 3 (120-5).
866
Kanovsky J
et al.
A new homozygous mutation of the KCNQ1 gene associated with both Romano-Ward and incomplete Jervell Lange-Nielsen syndromes in two sisters.
Heart Rhythm,
2010
Apr
, 7 (531-3).
867
Nof E
et al.
A common single nucleotide polymorphism can exacerbate long-QT type 2 syndrome leading to sudden infant death.
Circ Cardiovasc Genet,
2010
Apr
, 3 (199-206).
868
El Harchi A
et al.
Action potential clamp and mefloquine sensitivity of recombinant 'I KS' channels incorporating the V307L KCNQ1 mutation.
J. Physiol. Pharmacol.,
2010
Apr
, 61 (123-31).
869
Ohshige T
et al.
A single nucleotide polymorphism in KCNQ1 is associated with susceptibility to diabetic nephropathy in japanese subjects with type 2 diabetes.
Diabetes Care,
2010
Apr
, 33 (842-6).
870
Gladding PA
et al.
Posthumous diagnosis of long QT syndrome from neonatal screening cards.
Heart Rhythm,
2010
Apr
, 7 (481-6).
871
Subbiah RN
et al.
Torsades de pointes during complete atrioventricular block: Genetic factors and electrocardiogram correlates.
Can J Cardiol,
2010
Apr
, 26 (208-12).
872
Oh-McGinnis R
et al.
Rescue of placental phenotype in a mechanistic model of Beckwith-Wiedemann syndrome.
BMC Dev. Biol.,
2010
, 10 (50).
873
Duranton C
et al.
KCNQ1 K+ channels are involved in lipopolysaccharide-induced apoptosis of distal kidney cells.
Cell. Physiol. Biochem.,
2010
, 25 (367-78).
874
Tsai FJ
et al.
A genome-wide association study identifies susceptibility variants for type 2 diabetes in Han Chinese.
PLoS Genet.,
2010
, 6 (e1000847).
876
Frost JM
et al.
Telomeric NAP1L4 and OSBPL5 of the KCNQ1 cluster, and the DECORIN gene are not imprinted in human trophoblast stem cells.
PLoS ONE,
2010
, 5 (e11595).
877
Roepke TK
et al.
Targeted deletion of Kcne2 causes gastritis cystica profunda and gastric neoplasia.
PLoS ONE,
2010
, 5 (e11451).
878
Kwak SH
et al.
Polymorphisms in KCNQ1 are associated with gestational diabetes in a Korean population.
Horm Res Paediatr,
2010
, 74 (333-8).
879
Kotta CM
et al.
Cardiac ion channel gene mutations in Greek long QT syndrome patients.
J. Appl. Genet.,
2010
, 51 (515-8).
880
Nakajima T
et al.
Aborted cardiac arrest in a patient carrying KCNE1 D85N variant during the postpartum period.
Intern. Med.,
2010
, 49 (1875-8).
881
Cardona K
et al.
Effects of late sodium current enhancement during LQT-related arrhythmias. A simulation study.
Conf Proc IEEE Eng Med Biol Soc,
2010
, 2010 (3237-40).
882
Chen Z
et al.
KCNQ1 gene polymorphisms are associated with lipid parameters in a Chinese Han population.
Cardiovasc Diabetol,
2010
, 9 (35).
883
Nakajima T
et al.
Post myocardial ischemia-associated torsades de pointes in a patient carrying a KCNQ1 G643S variant.
Intern. Med.,
2010
, 49 (2587-91).
884
Lee HA
et al.
Cellular mechanism of the QT prolongation induced by sulpiride.
Int. J. Toxicol.,
2009 May-Jun
, 28 (207-12).
885
Strutz-Seebohm N
et al.
Serum- and glucocorticoid-inducible kinases (SGK) regulate KCNQ1/KCNE potassium channels.
Channels (Austin),
2009 Mar-Apr
, 3 (88-90).
886
Towart R
et al.
Blockade of the I(Ks) potassium channel: an overlooked cardiovascular liability in drug safety screening?
J Pharmacol Toxicol Methods,
2009 Jul-Aug
, 60 (1-10).
887
Kurokawa J
et al.
KCNE variants reveal a critical role of the beta subunit carboxyl terminus in PKA-dependent regulation of the IKs potassium channel.
Channels (Austin),
2009 Jan-Feb
, 3 (16-24).
888
Qi Q
et al.
Common variants in KCNQ1 are associated with type 2 diabetes and impaired fasting glucose in a Chinese Han population.
Hum. Mol. Genet.,
2009
Sep
15
, 18 (3508-15).
889
Rieg T
et al.
Unravelling a role for KCNQ1 in K+ recycling and gastric acid secretion.
J. Physiol. (Lond.),
2009
Sep
1
, 587 (4149-50).
890
Guo J
et al.
Extracellular K+ concentration controls cell surface density of IKr in rabbit hearts and of the HERG channel in human cell lines.
J. Clin. Invest.,
2009
Sep
, 119 (2745-57).
891
Vanoye CG
et al.
Distinct subdomains of the KCNQ1 S6 segment determine channel modulation by different KCNE subunits.
J. Gen. Physiol.,
2009
Sep
, 134 (207-17).
892
Kapplinger JD
et al.
Spectrum and prevalence of mutations from the first 2,500 consecutive unrelated patients referred for the FAMILION long QT syndrome genetic test.
Heart Rhythm,
2009
Sep
, 6 (1297-303).
893
Overholser BR
et al.
Paroxysmal beta-adrenergic receptor-mediated alterations in ventricular repolarization at rapid heart rates during inhibition of delayed rectifier currents.
J. Cardiovasc. Pharmacol.,
2009
Sep
, 54 (253-62).
895
Heron SE
et al.
Neonatal seizures and Long QT Syndrome: A cardiocerebral channelopathy?
Epilepsia,
2009
Oct
27
, ().
896
Goldman AM
et al.
Arrhythmia in heart and brain: KCNQ1 mutations link epilepsy and sudden unexplained death.
Sci Transl Med,
2009
Oct
14
, 1 (2ra6).
897
Ziv O
et al.
Origin of complex behaviour of spatially discordant alternans in a transgenic rabbit model of type 2 long QT syndrome.
J. Physiol. (Lond.),
2009
Oct
1
, 587 (4661-80).
898
Roepke TK
et al.
Kcne2 deletion uncovers its crucial role in thyroid hormone biosynthesis.
Nat. Med.,
2009
Oct
, 15 (1186-94).
899
Jonsson A
et al.
A variant in the KCNQ1 gene predicts future type 2 diabetes and mediates impaired insulin secretion.
Diabetes,
2009
Oct
, 58 (2409-13).
900
Itoh H
et al.
Latent genetic backgrounds and molecular pathogenesis in drug-induced long-QT syndrome.
Circ Arrhythm Electrophysiol,
2009
Oct
, 2 (511-23).
901
Xu Q
et al.
Crystal structure of a trimeric form of the K(V)7.1 (KCNQ1) A-domain tail coiled-coil reveals structural plasticity and context dependent changes in a putative coiled-coil trimerization motif.
Protein Sci.,
2009
Oct
, 18 (2100-14).
902
Kapa S
et al.
Genetic testing for long-QT syndrome: distinguishing pathogenic mutations from benign variants.
Circulation,
2009
Nov
3
, 120 (1752-60).
903
Shimizu W
et al.
Genotype-phenotype aspects of type 2 long QT syndrome.
J. Am. Coll. Cardiol.,
2009
Nov
24
, 54 (2052-62).
904
Padilla K
et al.
The KCNQ2/3 selective channel opener ICA-27243 binds to a novel voltage-sensor domain site.
Neurosci. Lett.,
2009
Nov
13
, 465 (138-42).
905
O'Mahony F
et al.
Novel female sex-dependent actions of oestrogen in the intestine.
J. Physiol. (Lond.),
2009
Nov
1
, 587 (5039-44).
906
Brink PA
et al.
Of founder populations, long QT syndrome, and destiny.
Heart Rhythm,
2009
Nov
, 6 (S25-33).
907
Lang F
et al.
Targeting SGK1 in diabetes.
Expert Opin. Ther. Targets,
2009
Nov
, 13 (1303-11).
908
Hedley PL
et al.
The genetic basis of long QT and short QT syndromes: a mutation update.
Hum. Mutat.,
2009
Nov
, 30 (1486-511).
909
Ogawa K
et al.
Isolated non-compaction of the ventricular myocardium associated with long QT syndrome: a report of 2 cases.
Circ. J.,
2009
Nov
, 73 (2169-72).
910
Zhou Q
et al.
Association of KCNQ1 gene polymorphism with gestational diabetes mellitus in a Chinese population.
Diabetologia,
2009
Nov
, 52 (2466-8).
911
Maguy A
et al.
Ion channel subunit expression changes in cardiac Purkinje fibers: a potential role in conduction abnormalities associated with congestive heart failure.
Circ. Res.,
2009
May
8
, 104 (1113-22).
912
Li W
et al.
The G314S KCNQ1 mutation exerts a dominant-negative effect on expression of KCNQ1 channels in oocytes.
Biochem. Biophys. Res. Commun.,
2009
May
29
, 383 (206-9).
913
Bankston JR
et al.
Molecular determinants of local anesthetic action of beta-blocking drugs: Implications for therapeutic management of long QT syndrome variant 3.
J. Mol. Cell. Cardiol.,
2009
May
28
, ().
914
Hammami S
et al.
Cell volume and membrane stretch independently control K+ channel activity.
J. Physiol. (Lond.),
2009
May
15
, 587 (2225-31).
915
Makita N
Phenotypic overlap of cardiac sodium channelopathies: individual-specific or mutation-specific?
Circ. J.,
2009
May
, 73 (810-7).
916
Nishio H
et al.
Identification of an ethnic-specific variant (V207M) of the KCNQ1 cardiac potassium channel gene in sudden unexplained death and implications from a knock-in mouse model.
Int. J. Legal Med.,
2009
May
, 123 (253-7).
917
Matavel A
et al.
PKC activation and PIP(2) depletion underlie biphasic regulation of IKs by Gq-coupled receptors.
J. Mol. Cell. Cardiol.,
2009
May
, 46 (704-12).
918
Chen J
et al.
PKA phosphorylation of HERG protein regulates the rate of channel synthesis.
Am. J. Physiol. Heart Circ. Physiol.,
2009
May
, 296 (H1244-54).
919
Huang H
et al.
Biophysical characterization of a new SCN5A mutation S1333Y in a SIDS infant linked to long QT syndrome.
FEBS Lett.,
2009
Mar
4
, 583 (890-6).
920
Yeh YH
et al.
Funny current downregulation and sinus node dysfunction associated with atrial tachyarrhythmia: a molecular basis for tachycardia-bradycardia syndrome.
Circulation,
2009
Mar
31
, 119 (1576-85).
921
Tsurugi T
et al.
Differential modulation of late sodium current by protein kinase A in R1623Q mutant of LQT3.
Life Sci.,
2009
Mar
13
, 84 (380-7).
922
McCrossan ZA
et al.
Regulation of the Kv2.1 potassium channel by MinK and MiRP1.
J. Membr. Biol.,
2009
Mar
, 228 (1-14).
923
Salama G
et al.
Arrhythmia phenotype in mouse models of human long QT.
,
2009
Mar
, 24 (77-87).
924
Aurlien D
et al.
New SCN5A mutation in a SUDEP victim with idiopathic epilepsy.
,
2009
Mar
, 18 (158-60).
925
McCallum LA
et al.
Expression and function of K(v)7 channels in murine myometrium throughout oestrous cycle.
Pflugers Arch.,
2009
Mar
, 457 (1111-20).
926
Namkung W
et al.
In situ measurement of airway surface liquid [K+] using a ratioable K+-sensitive fluorescent dye.
J. Biol. Chem.,
2009
Jun
5
, 284 (15916-26).
927
Ureche ON
et al.
Differential modulation of cardiac potassium channels by Grb adaptor proteins.
Biochem. Biophys. Res. Commun.,
2009
Jun
19
, 384 (28-31).
928
Jiang M
et al.
Dynamic partnership between KCNQ1 and KCNE1 and influence on cardiac IKs current amplitude by KCNE2.
J. Biol. Chem.,
2009
Jun
12
, 284 (16452-62).
929
Ahrens-Nicklas RC
et al.
Re-evaluating the efficacy of beta-adrenergic agonists and antagonists in long QT-3 syndrome through computational modelling.
Cardiovasc. Res.,
2009
Jun
1
, 82 (439-47).
930
Hardman RM
et al.
Ether-à-go-go-related gene K+ channels contribute to threshold excitability of mouse auditory brainstem neurons.
J. Physiol. (Lond.),
2009
Jun
1
, 587 (2487-97).
931
Xu X
et al.
MinK-dependent internalization of the IKs potassium channel.
Cardiovasc. Res.,
2009
Jun
1
, 82 (430-8).
932
Schimpf R
et al.
Channelopathies: Brugada syndrome, long QT syndrome, short QT syndrome, and CPVT.
,
2009
Jun
, 34 (281-8).
933
Zienciuk A
et al.
[Atrial fibrillation in the long QT syndrome]
Kardiol Pol,
2009
Jun
, 67 (681-4, discussion 685-6).
934
Bush WS
et al.
Genetic variation in the rhythmonome: ethnic variation and haplotype structure in candidate genes for arrhythmias.
Pharmacogenomics,
2009
Jun
, 10 (1043-53).
935
Tan JT
et al.
Genetic variation in KCNQ1 associates with fasting glucose and beta-cell function: a study of 3,734 subjects comprising three ethnicities living in Singapore.
Diabetes,
2009
Jun
, 58 (1445-9).
936
Boini KM
et al.
Enhanced insulin sensitivity of gene-targeted mice lacking functional KCNQ1.
Am. J. Physiol. Regul. Integr. Comp. Physiol.,
2009
Jun
, 296 (R1695-701).
937
Sun Y
et al.
A novel nonsense mutation Y652X in the S6/pore region of human ether-go-go gene found in a long QT syndrome family.
Scand. Cardiovasc. J.,
2009
Jun
, 43 (181-6).
938
Silva JR
et al.
A multiscale model linking ion-channel molecular dynamics and electrostatics to the cardiac action potential.
Proc. Natl. Acad. Sci. U.S.A.,
2009
Jul
7
, 106 (11102-6).
939
Haitin Y
et al.
Intracellular domains interactions and gated motions of I(KS) potassium channel subunits.
EMBO J.,
2009
Jul
22
, 28 (1994-2005).
940
Bajwa PJ
et al.
Activation of PPARgamma by rosiglitazone attenuates intestinal Cl- secretion.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2009
Jul
, 297 (G82-9).
941
Hu C
et al.
Variations in KCNQ1 are associated with type 2 diabetes and beta cell function in a Chinese population.
Diabetologia,
2009
Jul
, 52 (1322-5).
942
Zankov DP
et al.
Adrenergic regulation of the rapid component of delayed rectifier K+ current: implications for arrhythmogenesis in LQT2 patients.
Heart Rhythm,
2009
Jul
, 6 (1038-46).
943
Takeuchi F
et al.
Confirmation of multiple risk Loci and genetic impacts by a genome-wide association study of type 2 diabetes in the Japanese population.
Diabetes,
2009
Jul
, 58 (1690-9).
944
Müssig K
et al.
Association of type 2 diabetes candidate polymorphisms in KCNQ1 with incretin and insulin secretion.
Diabetes,
2009
Jul
, 58 (1715-20).
945
Pawelczyk M
et al.
Analysis of gene polymorphisms associated with K ion circulation in the inner ear of patients susceptible and resistant to noise-induced hearing loss.
Ann. Hum. Genet.,
2009
Jul
, 73 (411-21).
946
Missan S
et al.
Regulation of wild-type and mutant KCNQ1/KCNE1 channels by tyrosine kinase.
Pflugers Arch.,
2009
Jul
, 458 (471-80).
947
El-Sherif N
et al.
Role of pharmacotherapy in cardiac ion channelopathies.
,
2009
Jul
, 7 (358-66).
948
Dautova Y
et al.
Atrial arrhythmogenesis in wild-type and Scn5a+/delta murine hearts modelling LQT3 syndrome.
Pflugers Arch.,
2009
Jul
, 458 (443-57).
949
Ackerman MJ
State of postmortem genetic testing known as the cardiac channel molecular autopsy in the forensic evaluation of unexplained sudden cardiac death in the young.
Pacing Clin Electrophysiol,
2009
Jul
, 32 Suppl 2 (S86-9).
950
Liu Y
et al.
Variants in KCNQ1 are associated with susceptibility to type 2 diabetes in the population of mainland China.
Diabetologia,
2009
Jul
, 52 (1315-21).
951
Yang T
et al.
Probing the mechanisms underlying modulation of quinidine sensitivity to cardiac I(Ks) block by protein kinase A-mediated I(Ks) phosphorylation.
Br. J. Pharmacol.,
2009
Jul
, 157 (952-61).
952
Zamorano-León JJ
et al.
Novel mutation (H402R) in the S1 domain of KCNH2-encoded gene associated with long QT syndrome in a Spanish family.
Int. J. Cardiol.,
2009
Jan
9
, ().
953
Delisle BP
et al.
Small GTPase determinants for the Golgi processing and plasmalemmal expression of human ether-a-go-go related (hERG) K+ channels.
J. Biol. Chem.,
2009
Jan
30
, 284 (2844-53).
954
Chung DY
et al.
Location of KCNE1 relative to KCNQ1 in the I(KS) potassium channel by disulfide cross-linking of substituted cysteines.
Proc. Natl. Acad. Sci. U.S.A.,
2009
Jan
20
, 106 (743-8).
955
Wang H
et al.
Expression of the voltage-gated potassium channel KCNQ1 in mammalian taste bud cells and the effect of its null-mutation on taste preferences.
J. Comp. Neurol.,
2009
Jan
20
, 512 (384-98).
956
Johnson JN
et al.
Identification of a possible pathogenic link between congenital long QT syndrome and epilepsy.
Neurology,
2009
Jan
20
, 72 (224-31).
957
Ikrar T
et al.
Evaluation of channel function after alteration of amino acid residues at the pore center of KCNQ1 channel.
Biochem. Biophys. Res. Commun.,
2009
Jan
16
, 378 (589-94).
958
Manderfield LJ
et al.
KCNE4 domains required for inhibition of KCNQ1.
J. Physiol. (Lond.),
2009
Jan
15
, 587 (303-14).
959
Barajas-Martinez H
et al.
Larger dispersion of INa in female dog ventricle as a mechanism for gender-specific incidence of cardiac arrhythmias.
Cardiovasc. Res.,
2009
Jan
1
, 81 (82-9).
960
Shin JM
et al.
The gastric HK-ATPase: structure, function, and inhibition.
Pflugers Arch.,
2009
Jan
, 457 (609-22).
961
Lehtinen AB
et al.
Relationship between genetic variants in myocardial sodium and potassium channel genes and QT interval duration in diabetics: the Diabetes Heart Study.
Ann Noninvasive Electrocardiol,
2009
Jan
, 14 (72-9).
962
Markiewicz-Łoskot G
et al.
The risk of cardiac events and genotype-based management of LQTS patients.
Ann Noninvasive Electrocardiol,
2009
Jan
, 14 (86-92).
966
Diness JG
et al.
Antiarrhythmic effect of IKr activation in a cellular model of LQT3.
Heart Rhythm,
2009
Jan
, 6 (100-6).
967
Kauferstein S
et al.
Cardiac gene defects can cause sudden cardiac death in young people.
Dtsch Arztebl Int,
2009
Jan
, 106 (41-7).
968
Kanber D
et al.
Low frequency of imprinting defects in ICSI children born small for gestational age.
Eur. J. Hum. Genet.,
2009
Jan
, 17 (22-9).
969
Asada K
et al.
Redox- and calmodulin-dependent S-nitrosylation of the KCNQ1 channel.
J. Biol. Chem.,
2009
Feb
27
, 284 (6014-20).
970
Li W
et al.
Congenital long QT syndrome caused by the F275S KCNQ1 mutation: mechanism of impaired channel function.
Biochem. Biophys. Res. Commun.,
2009
Feb
27
, 380 (127-31).
971
Peroz D
et al.
LQT1-associated mutations increase KCNQ1 proteasomal degradation independently of Derlin-1.
J. Biol. Chem.,
2009
Feb
20
, 284 (5250-6).
972
Yang HT
et al.
HERG -F463L Potassium Channels Linked to Long QT Syndrome Reduce I Current by a Trafficking-Deficient Mechanism.
Clin. Exp. Pharmacol. Physiol.,
2009
Feb
10
, ().
973
Bardou O
et al.
Molecular diversity and function of K+ channels in airway and alveolar epithelial cells.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2009
Feb
, 296 (L145-55).
974
Bruce S
et al.
Clinically distinct epigenetic subgroups in Silver-Russell syndrome: the degree of H19 hypomethylation associates with phenotype severity and genital and skeletal anomalies.
J. Clin. Endocrinol. Metab.,
2009
Feb
, 94 (579-87).
975
Ma L
et al.
[The effects of hypokalemia on the Na+ channel in cardiac tissue--a computer simulation study]
Sheng Wu Yi Xue Gong Cheng Xue Za Zhi,
2009
Feb
, 26 (1-5).
976
Potet F
et al.
Genetic screening in C. elegans identifies rho-GTPase activating protein 6 as novel HERG regulator.
J. Mol. Cell. Cardiol.,
2009
Feb
, 46 (257-67).
977
Pan N
et al.
A hydrophobicity-dependent motif responsible for surface expression of cardiac potassium channel.
Cell. Signal.,
2009
Feb
, 21 (349-55).
978
Greenwood IA
et al.
KCNQ-encoded channels regulate Na+ transport across H441 lung epithelial cells.
Pflugers Arch.,
2009
Feb
, 457 (785-94).
979
Crotti L
et al.
A KCNH2 branch point mutation causing aberrant splicing contributes to an explanation of genotype-negative long QT syndrome.
Heart Rhythm,
2009
Feb
, 6 (212-8).
980
Choi E
et al.
A shared mechanism for lipid- and {beta}-subunit-coordinated stabilization of the activated K+ channel voltage sensor.
,
2009
Dec
29
, ().
981
Lundby A
et al.
Structural basis for KV7.1/KCNEx interactions in the IKs channel complex.
,
2009
Dec
24
, ().
982
Sato A
et al.
Novel mechanisms of trafficking defect caused by KCNQ1 mutations found in long QT syndrome.
J. Biol. Chem.,
2009
Dec
11
, 284 (35122-33).
983
984
Winbo A
et al.
Low incidence of sudden cardiac death in a Swedish Y111C type 1 long-QT syndrome population.
Circ Cardiovasc Genet,
2009
Dec
, 2 (558-64).
985
Shi RM
et al.
[Gene mutation analysis of a Chinese family of congenital long Q-T syndrome type three.]
Zhonghua Er Ke Za Zhi,
2009
Dec
, 47 (926-30).
986
Iturralde-Torres P
et al.
[Genetic in long QT syndromes]
Arch Cardiol Mex,
2009
Dec
, 79 Suppl 2 (26-30).
987
Biliczki P
et al.
Trafficking-deficient long QT syndrome mutation KCNQ1-T587M confers severe clinical phenotype by impairment of KCNH2 membrane localization: evidence for clinically significant IKr-IKs alpha-subunit interaction.
Heart Rhythm,
2009
Dec
, 6 (1792-801).
988
Gustina AS
et al.
A recombinant N-terminal domain fully restores deactivation gating in N-truncated and long QT syndrome mutant hERG potassium channels.
Proc. Natl. Acad. Sci. U.S.A.,
2009
Aug
4
, 106 (13082-7).
989
Nishio Y
et al.
D85N, a KCNE1 polymorphism, is a disease-causing gene variant in long QT syndrome.
J. Am. Coll. Cardiol.,
2009
Aug
25
, 54 (812-9).
990
Omichi C
et al.
Congenital long QT syndrome presenting with a history of epilepsy: Misdiagnosis or relationship between channelopathies of the heart and brain?
Epilepsia,
2009
Aug
19
, ().
991
Song P
et al.
KCNQ1 is the luminal K+ recycling channel during stimulation of gastric acid secretion.
J. Physiol. (Lond.),
2009
Aug
1
, 587 (3955-65).
992
Yang T
et al.
Biophysical properties of 9 KCNQ1 mutations associated with long-QT syndrome.
Circ Arrhythm Electrophysiol,
2009
Aug
, 2 (417-26).
994
Das S
et al.
Mutation in the S3 segment of KCNQ1 results in familial lone atrial fibrillation.
Heart Rhythm,
2009
Aug
, 6 (1146-53).
995
Keller DI
et al.
Characterization of novel KCNH2 mutations in type 2 long QT syndrome manifesting as seizures.
Can J Cardiol,
2009
Aug
, 25 (455-62).
996
Lindegger N
et al.
Diastolic transient inward current in long QT syndrome type 3 is caused by Ca2+ overload and inhibited by ranolazine.
J. Mol. Cell. Cardiol.,
2009
Aug
, 47 (326-34).
997
Jons C
et al.
Mutations in conserved amino acids in the KCNQ1 channel and risk of cardiac events in type-1 long-QT syndrome.
J. Cardiovasc. Electrophysiol.,
2009
Aug
, 20 (859-65).
998
Terrenoire C
et al.
The cardiac IKs potassium channel macromolecular complex includes the phosphodiesterase PDE4D3.
J. Biol. Chem.,
2009
Apr
3
, 284 (9140-6).
999
Raviña T
et al.
Isoproterenol enhancement of I(Ks) current in amiodarone-induced long QT syndrome.
Int. J. Cardiol.,
2009
Apr
17
, 133 (402-6).
1000
Hayashi K
et al.
Long QT syndrome and associated gene mutation carriers in Japanese children: Results from ECG screening examination.
Clin. Sci.,
2009
Apr
16
, ().
1001
Khatami M
et al.
Accumulation of mitochondrial genome variations in Persian LQTS patients: a possible risk factor?
Cardiovasc. Pathol.,
2009
Apr
13
, ().
1002
Biswas S
et al.
Calcium-mediated dual-mode regulation of cardiac sodium channel gating.
Circ. Res.,
2009
Apr
10
, 104 (870-8).
1003
Nishio H
et al.
Postmortem molecular analysis for fatal arrhythmogenic disease in sudden unexplained death.
Leg Med (Tokyo),
2009
Apr
, 11 Suppl 1 (S119-20).
1004
Marjamaa A
et al.
Common candidate gene variants are associated with QT interval duration in the general population.
J. Intern. Med.,
2009
Apr
, 265 (448-58).
1005
Bardou O
et al.
[K+ channels and lung epithelial physiology]
Med Sci (Paris),
2009
Apr
, 25 (391-7).
1006
Joshi S
et al.
KCNQ modulators reveal a key role for KCNQ potassium channels in regulating the tone of rat pulmonary artery smooth muscle.
J. Pharmacol. Exp. Ther.,
2009
Apr
, 329 (368-76).
1007
Dai S
et al.
Supramolecular assemblies and localized regulation of voltage-gated ion channels.
Physiol. Rev.,
2009
Apr
, 89 (411-52).
1008
Yao Y
et al.
Aminoglycoside antibiotics restore functional expression of truncated HERG channels produced by nonsense mutations.
,
2009
Apr
, 6 (553-60).
1009
Brown DA
et al.
Neural KCNQ (Kv7) channels.
Br. J. Pharmacol.,
2009
Apr
, 156 (1185-95).
1010
Ohno S
et al.
Novel KCNE3 mutation reduces repolarizing potassium current and associated with long QT syndrome.
Hum. Mutat.,
2009
Apr
, 30 (557-63).
1011
Pfeufer A
et al.
Common variants at ten loci modulate the QT interval duration in the QTSCD Study.
Nat. Genet.,
2009
Apr
, 41 (407-14).
1012
Newton-Cheh C
et al.
Common variants at ten loci influence QT interval duration in the QTGEN Study.
Nat. Genet.,
2009
Apr
, 41 (399-406).
1013
Alders M
et al.
Determination of KCNQ1OT1 and H19 methylation levels in BWS and SRS patients using methylation-sensitive high-resolution melting analysis.
Eur. J. Hum. Genet.,
2009
Apr
, 17 (467-73).
1014
Jin Z
et al.
Expression and localization of K channels KCNQ2 and KCNQ3 in the mammalian cochlea.
Audiol. Neurootol.,
2009
, 14 (98-105).
1016
Ng SH
et al.
Parental origin of chromosomes influences crossover activity within the Kcnq1 transcriptionally imprinted domain of Mus musculus.
BMC Mol. Biol.,
2009
, 10 (43).
1017
Rotte A
et al.
APC sensitive gastric acid secretion.
Cell. Physiol. Biochem.,
2009
, 23 (133-42).
1018
Wickenden AD
et al.
Kv7 channels as targets for the treatment of pain.
Curr. Pharm. Des.,
2009
, 15 (1773-98).
1019
Mruk K
et al.
Discovery of a novel activator of KCNQ1-KCNE1 K channel complexes.
PLoS ONE,
2009
, 4 (e4236).
1020
Marjamaa A
et al.
High prevalence of four long QT syndrome founder mutations in the Finnish population.
Ann. Med.,
2009
, 41 (234-40).
1021
Chen J
et al.
Functional interactions between KCNE1 C-terminus and the KCNQ1 channel.
PLoS ONE,
2009
, 4 (e5143).
1022
Holmkvist J
et al.
The type 2 diabetes associated minor allele of rs2237895 KCNQ1 associates with reduced insulin release following an oral glucose load.
PLoS ONE,
2009
, 4 (e5872).
1023
Rotte A
et al.
Pioglitazone induced gastric acid secretion.
Cell. Physiol. Biochem.,
2009
, 24 (193-200).
1024
Roura-Ferrer M
et al.
Functional implications of KCNE subunit expression for the Kv7.5 (KCNQ5) channel.
Cell. Physiol. Biochem.,
2009
, 24 (325-34).
1025
Henrion U
et al.
Long QT syndrome-associated mutations in the voltage sensor of I(Ks) channels.
Cell. Physiol. Biochem.,
2009
, 24 (11-6).
1026
Blom SM
et al.
The acrylamide (S)-2 as a positive and negative modulator of Kv7 channels expressed in Xenopus laevis oocytes.
PLoS ONE,
2009
, 4 (e8251).
1027
Herlyn H
et al.
Positive selection at codon 38 of the human KCNE1 (= minK) gene and sporadic absence of 38Ser-coding mRNAs in Gly38Ser heterozygotes.
BMC Evol. Biol.,
2009
, 9 (188).
1028
Lei M
et al.
Genetic Na+ channelopathies and sinus node dysfunction.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (171-8).
1029
Grunnet M
et al.
hERG1 channel activators: a new anti-arrhythmic principle.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (347-62).
1030
Zimmer T
et al.
SCN5A channelopathies--an update on mutations and mechanisms.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (120-36).
1031
Hothi SS
et al.
Arrhythmogenic substrate and its modification by nicorandil in a murine model of long QT type 3 syndrome.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (267-80).
1032
Balijepalli RC
et al.
Caveolae, ion channels and cardiac arrhythmias.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (149-60).
1033
Bhuiyan ZA
et al.
An intronic mutation leading to incomplete skipping of exon-2 in KCNQ1 rescues hearing in Jervell and Lange-Nielsen syndrome.
Prog. Biophys. Mol. Biol.,
2008 Oct-Nov
, 98 (319-27).
1036
Christé G
et al.
Changes in action potentials and intracellular ionic homeostasis in a ventricular cell model related to a persistent sodium current in SCN5A mutations underlying LQT3.
Prog. Biophys. Mol. Biol.,
2008 Jan-Apr
, 96 (281-93).
1037
Zhang H
et al.
Repolarisation and vulnerability to re-entry in the human heart with short QT syndrome arising from KCNQ1 mutation--a simulation study.
Prog. Biophys. Mol. Biol.,
2008 Jan-Apr
, 96 (112-31).
1038
Hsueh CH
et al.
Functional studies on three novel HCNH2 mutations in Taiwan: identification of distinct mechanisms of channel defect and dissociation between glycosylation defect and assembly defect.
Biochem. Biophys. Res. Commun.,
2008
Sep
5
, 373 (572-8).
1039
Sale H
et al.
Physiological properties of hERG 1a/1b heteromeric currents and a hERG 1b-specific mutation associated with Long-QT syndrome.
Circ. Res.,
2008
Sep
26
, 103 (e81-95).
1040
Xiao L
et al.
Feedback remodeling of cardiac potassium current expression: a novel potential mechanism for control of repolarization reserve.
Circulation,
2008
Sep
2
, 118 (983-92).
1041
Panaghie G
et al.
Voltage-dependent C-type inactivation in a constitutively open K+ channel.
Biophys. J.,
2008
Sep
15
, 95 (2759-78).
1042
Restier L
et al.
Mechanisms by which atrial fibrillation-associated mutations in the S1 domain of KCNQ1 slow deactivation of IKs channels.
J. Physiol. (Lond.),
2008
Sep
1
, 586 (4179-91).
1043
Yeung S
et al.
Bimodal effects of the Kv7 channel activator retigabine on vascular K+ currents.
Br. J. Pharmacol.,
2008
Sep
, 155 (62-72).
1044
Molokhia M
et al.
Case ascertainment and estimated incidence of drug-induced long-QT syndrome: study in Southwest France.
,
2008
Sep
, 66 (386-95).
1045
Dai DZ
et al.
Induced ion currents and the endothelin pathway as targets for anti-arrhythmic agents.
,
2008
Sep
, 9 (1001-8).
1046
Khoueiry R
et al.
Dynamic CpG methylation of the KCNQ1OT1 gene during maturation of human oocytes.
J. Med. Genet.,
2008
Sep
, 45 (583-8).
1047
Eddy CA
et al.
Identification of large gene deletions and duplications in KCNQ1 and KCNH2 in patients with long QT syndrome.
Heart Rhythm,
2008
Sep
, 5 (1275-81).
1048
Yasuda K
et al.
Variants in KCNQ1 are associated with susceptibility to type 2 diabetes mellitus.
Nat. Genet.,
2008
Sep
, 40 (1092-7).
1049
Unoki H
et al.
SNPs in KCNQ1 are associated with susceptibility to type 2 diabetes in East Asian and European populations.
Nat. Genet.,
2008
Sep
, 40 (1098-102).
1050
Morokuma J
et al.
Modulation of potassium channel function confers a hyperproliferative invasive phenotype on embryonic stem cells.
Proc. Natl. Acad. Sci. U.S.A.,
2008
Oct
28
, 105 (16608-13).
1051
Pandey RR
et al.
Kcnq1ot1 antisense noncoding RNA mediates lineage-specific transcriptional silencing through chromatin-level regulation.
Mol. Cell,
2008
Oct
24
, 32 (232-46).
1052
Makiyama T
et al.
A novel SCN5A gain-of-function mutation M1875T associated with familial atrial fibrillation.
J. Am. Coll. Cardiol.,
2008
Oct
14
, 52 (1326-34).
1053
Roepke TK
et al.
Targeted deletion of kcne2 impairs ventricular repolarization via disruption of I(K,slow1) and I(to,f).
FASEB J.,
2008
Oct
, 22 (3648-60).
1054
Zeng H
et al.
1-[1-Hexyl-6-(methyloxy)-1H-indazol-3-yl]-2-methyl-1-propanone, a potent and highly selective small molecule blocker of the large-conductance voltage-gated and calcium-dependent K+ channel.
J. Pharmacol. Exp. Ther.,
2008
Oct
, 327 (168-77).
1055
O'Mahony F
et al.
Sex and estrous cycle-dependent rapid protein kinase signaling actions of estrogen in distal colonic cells.
Steroids,
2008
Oct
, 73 (889-94).
1056
Yasuda K
et al.
Clinical and electrophysiological features of Japanese pediatric long QT syndrome patients with KCNQ1 mutations.
Pediatr Int,
2008
Oct
, 50 (611-4).
1057
Yontar OC
et al.
Short QT syndrome: a very rare arrhythmogenic entity.
Acta Cardiol,
2008
Oct
, 63 (553-5).
1058
Benito B
et al.
A mutation in the sodium channel is responsible for the association of long QT syndrome and familial atrial fibrillation.
Heart Rhythm,
2008
Oct
, 5 (1434-40).
1059
Bal M
et al.
Homomeric and heteromeric assembly of KCNQ (Kv7) K+ channels assayed by total internal reflection fluorescence/fluorescence resonance energy transfer and patch clamp analysis.
J. Biol. Chem.,
2008
Nov
7
, 283 (30668-76).
1061
Mackie AR
et al.
Cardiovascular KCNQ (Kv7) potassium channels: physiological regulators and new targets for therapeutic intervention.
Mol. Pharmacol.,
2008
Nov
, 74 (1171-9).
1062
Otagiri T
et al.
Cardiac ion channel gene mutations in sudden infant death syndrome.
Pediatr. Res.,
2008
Nov
, 64 (482-7).
1063
Imredy JP
et al.
Modeling of the adrenergic response of the human IKs current (hKCNQ1/hKCNE1) stably expressed in HEK-293 cells.
Am. J. Physiol. Heart Circ. Physiol.,
2008
Nov
, 295 (H1867-81).
1064
Trinh NT
et al.
EGF and K+ channel activity control normal and cystic fibrosis bronchial epithelia repair.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2008
Nov
, 295 (L866-80).
1065
1066
Lin MT
et al.
In utero onset of long QT syndrome with atrioventricular block and spontaneous or lidocaine-induced ventricular tachycardia: compound effects of hERG pore region mutation and SCN5A N-terminus variant.
Heart Rhythm,
2008
Nov
, 5 (1567-74).
1067
Christé G
et al.
A new C-terminal hERG mutation A915fs+47X associated with symptomatic LQT2 and auditory-trigger syncope.
,
2008
Nov
, 5 (1577-86).
1068
Tenenbaum M
et al.
Identification of the gene causing long QT syndrome in an Israeli family.
Isr. Med. Assoc. J.,
2008
Nov
, 10 (809-11).
1069
Roura-Ferrer M
et al.
Skeletal muscle Kv7 (KCNQ) channels in myoblast differentiation and proliferation.
Biochem. Biophys. Res. Commun.,
2008
May
16
, 369 (1094-7).
1070
Schimpf R
et al.
Clinical and molecular genetics of the short QT syndrome.
Curr. Opin. Cardiol.,
2008
May
, 23 (192-8).
1071
Mackie AR
et al.
Vascular KCNQ potassium channels as novel targets for the control of mesenteric artery constriction by vasopressin, based on studies in single cells, pressurized arteries, and in vivo measurements of mesenteric vascular resistance.
J. Pharmacol. Exp. Ther.,
2008
May
, 325 (475-83).
1072
Ohno S
et al.
A novel mutation associated with Jervell and Lange-Nielsen syndrome in a Japanese family.
Circ. J.,
2008
May
, 72 (687-93).
1073
Schwartz PJ
et al.
Ion channel diseases in children: manifestations and management.
Curr. Opin. Cardiol.,
2008
May
, 23 (184-91).
1074
Peitersen T
et al.
Computational analysis of the effects of the hERG channel opener NS1643 in a human ventricular cell model.
,
2008
May
, 5 (734-41).
1075
Ikrar T
et al.
A double-point mutation in the selectivity filter site of the KCNQ1 potassium channel results in a severe phenotype, LQT1, of long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2008
May
, 19 (541-9).
1076
Johnson JN
et al.
Prevalence of early-onset atrial fibrillation in congenital long QT syndrome.
Heart Rhythm,
2008
May
, 5 (704-9).
1077
Schwartz PJ
et al.
Neural control of heart rate is an arrhythmia risk modifier in long QT syndrome.
J. Am. Coll. Cardiol.,
2008
Mar
4
, 51 (920-9).
1078
Raviña T
et al.
Acquired long QT syndrome: Unequal regression of Amiodarone-induced repolarization lengthening.
Int. J. Cardiol.,
2008
Mar
14
, 124 (395-9).
1079
Surber R
et al.
Combination of cardiac conduction disease and long QT syndrome caused by mutation T1620K in the cardiac sodium channel.
Cardiovasc. Res.,
2008
Mar
1
, 77 (740-8).
1080
Saint DA
The cardiac persistent sodium current: an appealing therapeutic target?
Br. J. Pharmacol.,
2008
Mar
, 153 (1133-42).
1081
Ravn LS
et al.
Gain of function in IKs secondary to a mutation in KCNE5 associated with atrial fibrillation.
Heart Rhythm,
2008
Mar
, 5 (427-35).
1082
Wu DZ
et al.
Palmatine, a protoberberine alkaloid, inhibits both Ca(2+)- and cAMP-activated Cl(-) secretion in isolated rat distal colon.
Br. J. Pharmacol.,
2008
Mar
, 153 (1203-13).
1083
Luo X
et al.
Genomic structure, transcriptional control, and tissue distribution of HERG1 and KCNQ1 genes.
Am. J. Physiol. Heart Circ. Physiol.,
2008
Mar
, 294 (H1371-80).
1084
Rhodes TE
et al.
Cardiac potassium channel dysfunction in sudden infant death syndrome.
J. Mol. Cell. Cardiol.,
2008
Mar
, 44 (571-81).
1085
Liu B
et al.
Antihistamine mepyramine directly inhibits KCNQ/M channel and depolarizes rat superior cervical ganglion neurons.
Neuropharmacology,
2008
Mar
, 54 (629-39).
1086
Manderfield LJ
et al.
KCNE4 can co-associate with the I(Ks) (KCNQ1-KCNE1) channel complex.
FEBS J.,
2008
Mar
, 275 (1336-49).
1087
Doolan A
et al.
Postmortem molecular analysis of KCNQ1 and SCN5A genes in sudden unexplained death in young Australians.
Int. J. Cardiol.,
2008
Jun
23
, 127 (138-41).
1088
Goldenberg I
et al.
Long QT syndrome.
J. Am. Coll. Cardiol.,
2008
Jun
17
, 51 (2291-300).
1089
Nakajo K
et al.
Second coiled-coil domain of KCNQ channel controls current expression and subfamily specific heteromultimerization by salt bridge networks.
J. Physiol. (Lond.),
2008
Jun
15
, 586 (2827-40).
1090
Jia L
et al.
Support vector machines classification of hERG liabilities based on atom types.
Bioorg. Med. Chem.,
2008
Jun
1
, 16 (6252-60).
1091
Brunner M
et al.
Mechanisms of cardiac arrhythmias and sudden death in transgenic rabbits with long QT syndrome.
J. Clin. Invest.,
2008
Jun
, 118 (2246-59).
1092
Xu X
et al.
KCNQ1 and KCNE1 in the IKs channel complex make state-dependent contacts in their extracellular domains.
J. Gen. Physiol.,
2008
Jun
, 131 (589-603).
1093
Makita N
et al.
The E1784K mutation in SCN5A is associated with mixed clinical phenotype of type 3 long QT syndrome.
J. Clin. Invest.,
2008
Jun
, 118 (2219-29).
1094
Mohammad F
et al.
Kcnq1ot1/Lit1 noncoding RNA mediates transcriptional silencing by targeting to the perinucleolar region.
Mol. Cell. Biol.,
2008
Jun
, 28 (3713-28).
1095
Hreiche R
et al.
Drug-induced long QT syndrome in women: review of current evidence and remaining gaps.
,
2008
Jun
, 5 (124-35).
1096
Crotti L
et al.
Gene symbol: KCNQ1. Disease: Long QT syndrome.
Hum. Genet.,
2008
Jun
, 123 (541).
1097
Crotti L
et al.
Gene symbol: KCNQ1. Disease: Long QT syndrome.
Hum. Genet.,
2008
Jun
, 123 (543).
1098
Ueda K
et al.
Syntrophin mutation associated with long QT syndrome through activation of the nNOS-SCN5A macromolecular complex.
Proc. Natl. Acad. Sci. U.S.A.,
2008
Jul
8
, 105 (9355-60).
1099
Tsai CT
et al.
Molecular genetics of atrial fibrillation.
J. Am. Coll. Cardiol.,
2008
Jul
22
, 52 (241-50).
1100
Diness TG
et al.
Antiarrhythmic properties of a rapid delayed-rectifier current activator in rabbit models of acquired long QT syndrome.
Cardiovasc. Res.,
2008
Jul
1
, 79 (61-9).
1101
Liu JF
et al.
Phenotypic variability in Caucasian and Japanese patients with matched LQT1 mutations.
Ann Noninvasive Electrocardiol,
2008
Jul
, 13 (234-41).
1102
Amin AS
et al.
Fever-induced QTc prolongation and ventricular arrhythmias in individuals with type 2 congenital long QT syndrome.
J. Clin. Invest.,
2008
Jul
, 118 (2552-61).
1103
Kuwamura M
et al.
Pancreatic metaplasia in the gastro-achlorhydria in WTC-dfk rat, a potassium channel Kcnq1 mutant.
Vet. Pathol.,
2008
Jul
, 45 (586-91).
1104
Arbour L
et al.
A KCNQ1 V205M missense mutation causes a high rate of long QT syndrome in a First Nations community of northern British Columbia: a community-based approach to understanding the impact.
Genet. Med.,
2008
Jul
, 10 (545-50).
1105
Shimizu W
Genetics of congenital long QT syndrome and Brugada syndrome.
Future Cardiol,
2008
Jul
, 4 (379-89).
1106
Shin JY
et al.
Two distinct mechanisms of silencing by the KvDMR1 imprinting control region.
EMBO J.,
2008
Jan
9
, 27 (168-78).
1107
Albert CM
et al.
Cardiac sodium channel gene variants and sudden cardiac death in women.
Circulation,
2008
Jan
1
, 117 (16-23).
1108
Rocheleau JM
et al.
KCNE peptides differently affect voltage sensor equilibrium and equilibration rates in KCNQ1 K+ channels.
J. Gen. Physiol.,
2008
Jan
, 131 (59-68).
1109
Sabir IN
et al.
Criteria for arrhythmogenicity in genetically-modified Langendorff-perfused murine hearts modelling the congenital long QT syndrome type 3 and the Brugada syndrome.
Pflugers Arch.,
2008
Jan
, 455 (637-51).
1110
Nehrke K
et al.
Intestinal Ca2+ wave dynamics in freely moving C. elegans coordinate execution of a rhythmic motor program.
Am. J. Physiol., Cell Physiol.,
2008
Jan
, 294 (C333-44).
1111
Gordon E
et al.
A KCNE2 mutation in a patient with cardiac arrhythmia induced by auditory stimuli and serum electrolyte imbalance.
Cardiovasc. Res.,
2008
Jan
, 77 (98-106).
1112
Vaglio M
et al.
A quantitative assessment of T-wave morphology in LQT1, LQT2, and healthy individuals based on Holter recording technology.
,
2008
Jan
, 5 (11-8).
1113
Jeyaraj D
et al.
I(Kr) channel blockade to unmask occult congenital long QT syndrome.
Heart Rhythm,
2008
Jan
, 5 (2-7).
1114
Morin TJ
et al.
Counting membrane-embedded KCNE beta-subunits in functioning K+ channel complexes.
Proc. Natl. Acad. Sci. U.S.A.,
2008
Feb
5
, 105 (1478-82).
1115
Wiener R
et al.
The KCNQ1 (Kv7.1) COOH terminus, a multitiered scaffold for subunit assembly and protein interaction.
J. Biol. Chem.,
2008
Feb
29
, 283 (5815-30).
1116
Moser SL
et al.
Multiple KCNQ potassium channel subtypes mediate basal anion secretion from the human airway epithelial cell line Calu-3.
J. Membr. Biol.,
2008
Feb
, 221 (153-63).
1117
Mancilla-Simbro C
et al.
Chlorthalidone inhibits the KvLQT1 potassium current in guinea-pig ventricular myocytes and oocytes from Xenopus laevis.
Br. J. Pharmacol.,
2008
Feb
, 153 (448-58).
1118
Miceli F
et al.
Molecular pharmacology and therapeutic potential of neuronal Kv7-modulating drugs.
,
2008
Feb
, 8 (65-74).
1119
Takemasa H
et al.
Coexistence of hERG current block and disruption of protein trafficking in ketoconazole-induced long QT syndrome.
Br. J. Pharmacol.,
2008
Feb
, 153 (439-47).
1120
Seebohm G
et al.
Long QT syndrome-associated mutations in KCNQ1 and KCNE1 subunits disrupt normal endosomal recycling of IKs channels.
Circ. Res.,
2008
Dec
5
, 103 (1451-7).
1121
Teng GQ
et al.
Homozygous missense N629D hERG (KCNH2) potassium channel mutation causes developmental defects in the right ventricle and its outflow tract and embryonic lethality.
Circ. Res.,
2008
Dec
5
, 103 (1483-91).
1122
Yang H
et al.
[Identification of a novel KCNH2 mutation in a family with congenital long QT syndrome and prediction of the secondary structure of its encoding protein]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2008
Dec
, 25 (704-7).
1123
Zienciuk A
et al.
[QTc duration in the long QT syndrome type 2 - diagnostic dilemma]
,
2008
Dec
, 66 (1333-7).
1124
Sazhenova EA
et al.
[Epimutations of the KCNQ1OT1 imprinting center of chromosome 11 in early human embryo lethality]
Genetika,
2008
Dec
, 44 (1609-16).
1125
Shimizu W
Clinical impact of genetic studies in lethal inherited cardiac arrhythmias.
Circ. J.,
2008
Dec
, 72 (1926-36).
1126
Odening KE
et al.
Pharmacogenomics of anesthetic drugs in transgenic LQT1 and LQT2 rabbits reveal genotype-specific differential effects on cardiac repolarization.
Am. J. Physiol. Heart Circ. Physiol.,
2008
Dec
, 295 (H2264-72).
1127
Zaika O
et al.
Determinants within the turret and pore-loop domains of KCNQ3 K+ channels governing functional activity.
Biophys. J.,
2008
Dec
, 95 (5121-37).
1128
Moss AJ
et al.
Ranolazine shortens repolarization in patients with sustained inward sodium current due to type-3 long-QT syndrome.
J. Cardiovasc. Electrophysiol.,
2008
Dec
, 19 (1289-93).
1129
Kang C
et al.
Structure of KCNE1 and implications for how it modulates the KCNQ1 potassium channel.
Biochemistry,
2008
Aug
5
, 47 (7999-8006).
1130
Sun DD
et al.
Tanshinone IIA: a new activator of human cardiac KCNQ1/KCNE1 (I(Ks)) potassium channels.
Eur. J. Pharmacol.,
2008
Aug
20
, 590 (317-21).
1131
Gao Z
et al.
Desensitization of chemical activation by auxiliary subunits: convergence of molecular determinants critical for augmenting KCNQ1 potassium channels.
J. Biol. Chem.,
2008
Aug
15
, 283 (22649-58).
1132
Nicolas CS
et al.
IKs response to protein kinase A-dependent KCNQ1 phosphorylation requires direct interaction with microtubules.
Cardiovasc. Res.,
2008
Aug
1
, 79 (427-35).
1133
Olszak-Waśkiewicz M
et al.
The association between SCN5A, KCNQ1 and KCNE1 gene polymorphisms and complex ventricular arrhythmias in survivors of myocardial infarction.
,
2008
Aug
, 66 (845-53; discussion 854-5).
1134
Huo J
et al.
The G604S-hERG mutation alters the biophysical properties and exerts a dominant-negative effect on expression of hERG channels in HEK293 cells.
Pflugers Arch.,
2008
Aug
, 456 (917-28).
1135
Webster G
et al.
Congenital long-QT syndromes: a clinical and genetic update from infancy through adulthood.
Trends Cardiovasc. Med.,
2008
Aug
, 18 (216-24).
1136
Wu G
et al.
alpha-1-syntrophin mutation and the long-QT syndrome: a disease of sodium channel disruption.
Circ Arrhythm Electrophysiol,
2008
Aug
, 1 (193-201).
1137
Peroz D
et al.
Kv7.1 (KCNQ1) properties and channelopathies.
J. Physiol. (Lond.),
2008
Apr
1
, 586 (1785-9).
1138
Maljevic S
et al.
Nervous system KV7 disorders: breakdown of a subthreshold brake.
J. Physiol. (Lond.),
2008
Apr
1
, 586 (1791-801).
1139
Siem G
et al.
Jervell and Lange-Nielsen syndrome in Norwegian children: aspects around cochlear implantation, hearing, and balance.
,
2008
Apr
, 29 (261-9).
1140
Lee WK
et al.
Evidence for KCNQ1 K+ channel expression in rat zymogen granule membranes and involvement in cholecystokinin-induced pancreatic acinar secretion.
Am. J. Physiol., Cell Physiol.,
2008
Apr
, 294 (C879-92).
1141
Kaufhold MA
et al.
Localization, trafficking, and significance for acid secretion of parietal cell Kir4.1 and KCNQ1 K+ channels.
Gastroenterology,
2008
Apr
, 134 (1058-69).
1142
Patel C
et al.
Pharmacological approach to the treatment of long and short QT syndromes.
Pharmacol. Ther.,
2008
Apr
, 118 (138-51).
1143
Haapalahti P
et al.
Electrocardiographic interventricular dispersion of repolarization during autonomic adaptation in LQT1 subtype of long QT syndrome.
Scand. Cardiovasc. J.,
2008
Apr
, 42 (130-6).
1144
Zhang Y
et al.
Single nucleotide polymorphisms and haplotype of four genes encoding cardiac ion channels in Chinese and their association with arrhythmia.
Ann Noninvasive Electrocardiol,
2008
Apr
, 13 (180-90).
1145
Remme CA
et al.
Cardiac sodium channel overlap syndromes: different faces of SCN5A mutations.
Trends Cardiovasc. Med.,
2008
Apr
, 18 (78-87).
1146
Thomas G
et al.
Pharmacological separation of early afterdepolarizations from arrhythmogenic substrate in DeltaKPQ Scn5a murine hearts modelling human long QT 3 syndrome.
Acta Physiol (Oxf),
2008
Apr
, 192 (505-17).
1147
Raudenská M
et al.
Mutation analysis of candidate genes SCN1B, KCND3 and ANK2 in patients with clinical diagnosis of long QT syndrome.
Physiol Res,
2008
, 57 (857-62).
1148
Jackson HA
et al.
Evolutionary analyses of KCNQ1 and HERG voltage-gated potassium channel sequences reveal location-specific susceptibility and augmented chemical severities of arrhythmogenic mutations.
BMC Evol. Biol.,
2008
, 8 (188).
1149
Drago A
et al.
Strategy for a genetic assessment of antipsychotic and antidepressant-related proarrhythmia.
Curr. Med. Chem.,
2008
, 15 (2472-517).
1150
Ager EI
et al.
Evolution of the CDKN1C-KCNQ1 imprinted domain.
BMC Evol. Biol.,
2008
, 8 (163).
1152
Zhang X
et al.
Protective effect of KCNH2 single nucleotide polymorphism K897T in LQTS families and identification of novel KCNQ1 and KCNH2 mutations.
BMC Med. Genet.,
2008
, 9 (87).
1153
Morokuma J
et al.
KCNQ1 and KCNE1 K+ channel components are involved in early left-right patterning in Xenopus laevis embryos.
Cell. Physiol. Biochem.,
2008
, 21 (357-72).
1154
Olszak-Waśkiewicz M
et al.
Novel KCNQ1 mutations in patients after myocardial infarction.
,
2008
, 15 (252-60).
1155
Berge KE
et al.
Molecular genetic analysis of long QT syndrome in Norway indicating a high prevalence of heterozygous mutation carriers.
Scand. J. Clin. Lab. Invest.,
2008
, 68 (362-8).
1156
Haitin Y
et al.
S1 constrains S4 in the voltage sensor domain of Kv7.1 K+ channels.
PLoS ONE,
2008
, 3 (e1935).
1157
Shamgar L
et al.
KCNE1 constrains the voltage sensor of Kv7.1 K+ channels.
PLoS ONE,
2008
, 3 (e1943).
1158
Zhang S
et al.
Identification of a novel KCNQ1 mutation associated with both Jervell and Lange-Nielsen and Romano-Ward forms of long QT syndrome in a Chinese family.
BMC Med. Genet.,
2008
, 9 (24).
1159
Delpón E
et al.
Functional Effects of KCNE3 Mutation and its Role in the Development of Brugada Syndrome.
,
2008
, 1 (209-218).
1160
Lee YH
et al.
Association between polymorphisms in SLC30A8, HHEX, CDKN2A/B, IGF2BP2, FTO, WFS1, CDKAL1, KCNQ1 and type 2 diabetes in the Korean population.
J. Hum. Genet.,
2008
, 53 (991-8).
1161
Abbott GW
et al.
Impact of ancillary subunits on ventricular repolarization.
,
2007 Nov-Dec
, 40 (S42-6).
1162
Lopes CM
et al.
Protein kinase A modulates PLC-dependent regulation and PIP2-sensitivity of K+ channels.
Channels (Austin),
2007 Mar-Apr
, 1 (124-34).
1163
Balijepalli RC
et al.
Kv11.1 (ERG1) K+ channels localize in cholesterol and sphingolipid enriched membranes and are modulated by membrane cholesterol.
Channels (Austin),
2007 Jul-Aug
, 1 (263-72).
1164
Bankston JR
et al.
A novel LQT-3 mutation disrupts an inactivation gate complex with distinct rate-dependent phenotypic consequences.
Channels (Austin),
2007 Jul-Aug
, 1 (273-80).
1165
Medeiros-Domingo A
et al.
[New perspectives in long QT syndrome]
Rev. Invest. Clin.,
2007 Jan-Feb
, 59 (57-72).
1166
Ruan Y
et al.
Gating properties of SCN5A mutations and the response to mexiletine in long-QT syndrome type 3 patients.
Circulation,
2007
Sep
4
, 116 (1137-44).
1167
Paoloni-Giacobino A
et al.
Conserved features of imprinted differentially methylated domains.
Gene,
2007
Sep
1
, 399 (33-45).
1168
Flores CA
et al.
Abolition of Ca2+-mediated intestinal anion secretion and increased stool dehydration in mice lacking the intermediate conductance Ca2+-dependent K+ channel Kcnn4.
J. Physiol. (Lond.),
2007
Sep
1
, 583 (705-17).
1169
Nakajo K
et al.
KCNE1 and KCNE3 stabilize and/or slow voltage sensing S4 segment of KCNQ1 channel.
J. Gen. Physiol.,
2007
Sep
, 130 (269-81).
1170
vanTol BL
et al.
Contribution of KCNQ1 to the regulatory volume decrease in the human mammary epithelial cell line MCF-7.
Am. J. Physiol., Cell Physiol.,
2007
Sep
, 293 (C1010-9).
1171
Aizawa Y
et al.
A novel mutation in KCNQ1 associated with a potent dominant negative effect as the basis for the LQT1 form of the long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2007
Sep
, 18 (972-7).
1172
Gouas L
et al.
Confirmation of associations between ion channel gene SNPs and QTc interval duration in healthy subjects.
Eur. J. Hum. Genet.,
2007
Sep
, 15 (974-9).
1173
Stathopoulos A
et al.
Overexpression of mouse IsK protein fused to green fluorescent protein induces apoptosis of human astroglioma cells.
Neurol. Res.,
2007
Sep
, 29 (628-31).
1174
Zhang Y
et al.
A missense mutation (G604S) in the S5/pore region of HERG causes long QT syndrome in a Chinese family with a high incidence of sudden unexpected death.
Eur. J. Pediatr.,
2007
Sep
, 166 (927-33).
1175
Akkerhuis JM
et al.
[Congenital long QT-syndrome: the cause of recurrent syncope and sudden death at a young age]
,
2007
Oct
27
, 151 (2357-64).
1176
Etheridge SP
et al.
Long QT syndrome in children in the era of implantable defibrillators.
J. Am. Coll. Cardiol.,
2007
Oct
2
, 50 (1335-40).
1177
Tian C
et al.
Preparation, functional characterization, and NMR studies of human KCNE1, a voltage-gated potassium channel accessory subunit associated with deafness and long QT syndrome.
Biochemistry,
2007
Oct
16
, 46 (11459-72).
1178
Chen H
et al.
Serial perturbation of MinK in IKs implies an alpha-helical transmembrane span traversing the channel corpus.
Biophys. J.,
2007
Oct
1
, 93 (2332-40).
1179
Lang F
et al.
Functional significance of channels and transporters expressed in the inner ear and kidney.
Am. J. Physiol., Cell Physiol.,
2007
Oct
, 293 (C1187-208).
1180
Trinh NT
et al.
Involvement of KATP and KvLQT1 K+ channels in EGF-stimulated alveolar epithelial cell repair processes.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2007
Oct
, 293 (L870-82).
1181
Trepakova ES
et al.
Application of PatchXpress planar patch clamp technology to the screening of new drug candidates for cardiac KCNQ1/KCNE1 (I Ks) activity.
,
2007
Oct
, 5 (617-27).
1182
Heitzmann D
et al.
No potassium, no acid: K+ channels and gastric acid secretion.
,
2007
Oct
, 22 (335-41).
1183
Hur DG
et al.
KCNQ1/KCNE1 K+ channel and P2Y4 receptor are co-expressed from the time of birth in the apical membrane of rat strial marginal cells.
,
2007
Oct
, (30-5).
1184
Chung SK
et al.
Long QT and Brugada syndrome gene mutations in New Zealand.
,
2007
Oct
, 4 (1306-14).
1185
Poulsen AN
et al.
The KCNE1 beta-subunit exerts a transient effect on the KCNQ1 K+ channel.
Biochem. Biophys. Res. Commun.,
2007
Nov
9
, 363 (133-9).
1186
Crotti L
et al.
The common long-QT syndrome mutation KCNQ1/A341V causes unusually severe clinical manifestations in patients with different ethnic backgrounds: toward a mutation-specific risk stratification.
Circulation,
2007
Nov
20
, 116 (2366-75).
1187
Cemerikic D
et al.
Absence of KCNQ1-dependent K+ fluxes in proximal tubular cells of frog kidney.
Comp. Biochem. Physiol., Part A Mol. Integr. Physiol.,
2007
Nov
, 148 (635-44).
1188
Dennis A
et al.
hERG channel trafficking: novel targets in drug-induced long QT syndrome.
Biochem. Soc. Trans.,
2007
Nov
, 35 (1060-3).
1189
Harmer SC
et al.
The role of abnormal trafficking of KCNE1 in long QT syndrome 5.
Biochem. Soc. Trans.,
2007
Nov
, 35 (1074-6).
1190
Varga A
et al.
Effect of experimental hypercholesterolaemia on K+ channel alpha-subunit mRNA levels in rabbit hearts.
Eur. J. Pharmacol.,
2007
May
7
, 562 (130-1).
1191
Paavonen KJ
et al.
Beta1-adrenergic receptor polymorphisms, QTc interval and occurrence of symptoms in type 1 of long QT syndrome.
Int. J. Cardiol.,
2007
May
31
, 118 (197-202).
1192
Kunz L
et al.
Ovarian acetylcholine and ovarian KCNQ channels: insights into cellular regulatory systems of steroidogenic granulosa cells.
Life Sci.,
2007
May
30
, 80 (2195-8).
1193
Moss AJ
et al.
Clinical aspects of type-1 long-QT syndrome by location, coding type, and biophysical function of mutations involving the KCNQ1 gene.
Circulation,
2007
May
15
, 115 (2481-9).
1195
Yang Y
et al.
Human KCNQ1 S140G mutation is associated with atrioventricular blocks.
,
2007
May
, 4 (611-8).
1196
Denjoy I
et al.
[The Jervell and Lange-Nielsen syndrome. Natural history, molecular basis and clinical outcome]
Arch Mal Coeur Vaiss,
2007
May
, 100 (359-64).
1197
Lehtonen A
et al.
Further evidence of inherited long QT syndrome gene mutations in antiarrhythmic drug-associated torsades de pointes.
Heart Rhythm,
2007
May
, 4 (603-7).
1198
Bossard F
et al.
NHE-RF1 protein rescues DeltaF508-CFTR function.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2007
May
, 292 (L1085-94).
1199
Ocorr K
et al.
KCNQ potassium channel mutations cause cardiac arrhythmias in Drosophila that mimic the effects of aging.
Proc. Natl. Acad. Sci. U.S.A.,
2007
Mar
6
, 104 (3943-8).
1200
Seebohm G
et al.
Regulation of endocytic recycling of KCNQ1/KCNE1 potassium channels.
Circ. Res.,
2007
Mar
16
, 100 (686-92).
1201
Nielsen NH
et al.
Mutations in the Kv1.5 channel gene KCNA5 in cardiac arrest patients.
Biochem. Biophys. Res. Commun.,
2007
Mar
16
, 354 (776-82).
1202
Tsuji K
et al.
Mechanistic basis for the pathogenesis of long QT syndrome associated with a common splicing mutation in KCNQ1 gene.
J. Mol. Cell. Cardiol.,
2007
Mar
, 42 (662-9).
1203
Bachmann O
et al.
Mechanisms of secretion-associated shrinkage and volume recovery in cultured rabbit parietal cells.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2007
Mar
, 292 (G711-7).
1204
Witchel HJ
The hERG potassium channel as a therapeutic target.
Expert Opin. Ther. Targets,
2007
Mar
, 11 (321-36).
1205
Matos JE
et al.
Role of cholinergic-activated KCa1.1 (BK), KCa3.1 (SK4) and KV7.1 (KCNQ1) channels in mouse colonic Cl- secretion.
Acta Physiol (Oxf),
2007
Mar
, 189 (251-8).
1206
Ohno S
et al.
N- and C-terminal KCNE1 mutations cause distinct phenotypes of long QT syndrome.
,
2007
Mar
, 4 (332-40).
1207
Eckhardt LL
et al.
KCNJ2 mutations in arrhythmia patients referred for LQT testing: a mutation T305A with novel effect on rectification properties.
Heart Rhythm,
2007
Mar
, 4 (323-9).
1208
Wu L
et al.
Induction of high STAT1 expression in transgenic mice with LQTS and heart failure.
Biochem. Biophys. Res. Commun.,
2007
Jun
29
, 358 (449-54).
1209
Schmitt N
et al.
The novel C-terminal KCNQ1 mutation M520R alters protein trafficking.
Biochem. Biophys. Res. Commun.,
2007
Jun
22
, 358 (304-10).
1210
Koo SH
et al.
Mutation screening in KCNQ1, HERG, KCNE1, KCNE2 and SCN5A genes in a long QT syndrome family.
Ann. Acad. Med. Singap.,
2007
Jun
, 36 (394-8).
1211
Lerche C
et al.
Chromanol 293B binding in KCNQ1 (Kv7.1) channels involves electrostatic interactions with a potassium ion in the selectivity filter.
Mol. Pharmacol.,
2007
Jun
, 71 (1503-11).
1212
Guo J
et al.
Identification of IKr and its trafficking disruption induced by probucol in cultured neonatal rat cardiomyocytes.
J. Pharmacol. Exp. Ther.,
2007
Jun
, 321 (911-20).
1213
Calloe K
et al.
KCNQ channels are involved in the regulatory volume decrease response in primary neonatal rat cardiomyocytes.
Biochim. Biophys. Acta,
2007
Jun
, 1773 (764-73).
1214
Flaim SN
et al.
Arrhythmogenic consequences of Na+ channel mutations in the transmurally heterogeneous mammalian left ventricle: analysis of the I1768V SCN5A mutation.
Heart Rhythm,
2007
Jun
, 4 (768-78).
1215
Gong Q
et al.
Nonsense mutations in hERG cause a decrease in mutant mRNA transcripts by nonsense-mediated mRNA decay in human long-QT syndrome.
Circulation,
2007
Jul
3
, 116 (17-24).
1216
Arnaout R
et al.
Zebrafish model for human long QT syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
2007
Jul
3
, 104 (11316-21).
1217
Morin TJ
et al.
A derivatized scorpion toxin reveals the functional output of heteromeric KCNQ1-KCNE K+ channel complexes.
ACS Chem. Biol.,
2007
Jul
20
, 2 (469-73).
1218
Medeiros-Domingo A
et al.
SCN4B-encoded sodium channel beta4 subunit in congenital long-QT syndrome.
Circulation,
2007
Jul
10
, 116 (134-42).
1219
Yeung SY
et al.
Molecular expression and pharmacological identification of a role for K(v)7 channels in murine vascular reactivity.
Br. J. Pharmacol.,
2007
Jul
, 151 (758-70).
1220
Saenen JB
et al.
A single hERG mutation underlying a spectrum of acquired and congenital long QT syndrome phenotypes.
J. Mol. Cell. Cardiol.,
2007
Jul
, 43 (63-72).
1221
Furukawa T
et al.
Regulation of cardiac ion channels via non-genomic action of sex steroid hormones: implication for the gender difference in cardiac arrhythmias.
Pharmacol. Ther.,
2007
Jul
, 115 (106-15).
1222
Guan FY
et al.
[HERG K+ channel, the target of anti-arrhythmias drugs]
Yao Xue Xue Bao,
2007
Jul
, 42 (687-91).
1223
Takagi T
et al.
Phenotypic analysis of vertigo 2 Jackson mice with a Kcnq1 potassium channel mutation.
Exp. Anim.,
2007
Jul
, 56 (295-300).
1224
Drake E
et al.
Brief review: anesthetic implications of long QT syndrome in pregnancy.
,
2007
Jul
, 54 (561-72).
1225
Medeiros-Domingo A
et al.
[Clinical and genetic characteristics of long QT syndrome]
Rev Esp Cardiol,
2007
Jul
, 60 (739-52).
1226
Tian XL
et al.
Optical mapping of ventricular arrhythmias in LQTS mice with SCN5A mutation N1325S.
Biochem. Biophys. Res. Commun.,
2007
Jan
26
, 352 (879-83).
1227
Sauer AJ
et al.
Long QT syndrome in adults.
J. Am. Coll. Cardiol.,
2007
Jan
23
, 49 (329-37).
1228
Arnestad M
et al.
Prevalence of long-QT syndrome gene variants in sudden infant death syndrome.
Circulation,
2007
Jan
23
, 115 (361-7).
1229
Ma KJ
et al.
Modulation of KCNQ1 current by atrial fibrillation-associated KCNE4 (145E/D) gene polymorphism.
Chin. Med. J.,
2007
Jan
20
, 120 (150-4).
1230
Tester DJ
et al.
Postmortem long QT syndrome genetic testing for sudden unexplained death in the young.
J. Am. Coll. Cardiol.,
2007
Jan
16
, 49 (240-6).
1231
Yong SL
et al.
Characterization of the cardiac sodium channel SCN5A mutation, N1325S, in single murine ventricular myocytes.
Biochem. Biophys. Res. Commun.,
2007
Jan
12
, 352 (378-83).
1232
Stokoe KS
et al.
Effects of flecainide and quinidine on arrhythmogenic properties of Scn5a+/Delta murine hearts modelling long QT syndrome 3.
J. Physiol. (Lond.),
2007
Jan
1
, 578 (69-84).
1233
Thomas G
et al.
Effects of L-type Ca2+ channel antagonism on ventricular arrhythmogenesis in murine hearts containing a modification in the Scn5a gene modelling human long QT syndrome 3.
J. Physiol. (Lond.),
2007
Jan
1
, 578 (85-97).
1234
London B
et al.
Dispersion of repolarization and refractoriness are determinants of arrhythmia phenotype in transgenic mice with long QT.
J. Physiol. (Lond.),
2007
Jan
1
, 578 (115-29).
1235
Carlquist JF
et al.
Gene mutations, atrial fibrillation, and the elusive cigar.
Clin. Pharmacol. Ther.,
2007
Jan
, 81 (26-8).
1236
Sato A
et al.
Aberrant DNA methylation of imprinted loci in superovulated oocytes.
Hum. Reprod.,
2007
Jan
, 22 (26-35).
1237
Peretz A
et al.
Pre- and postsynaptic activation of M-channels by a novel opener dampens neuronal firing and transmitter release.
J. Neurophysiol.,
2007
Jan
, 97 (283-95).
1238
Liu XS
et al.
Electrical remodeling in a canine model of ischemic cardiomyopathy.
Am. J. Physiol. Heart Circ. Physiol.,
2007
Jan
, 292 (H560-71).
1239
Vecchietti S
et al.
In silico assessment of Y1795C and Y1795H SCN5A mutations: implication for inherited arrhythmogenic syndromes.
Am. J. Physiol. Heart Circ. Physiol.,
2007
Jan
, 292 (H56-65).
1240
Zhang ZS
et al.
Sodium channel kinetic changes that produce Brugada syndrome or progressive cardiac conduction system disease.
Am. J. Physiol. Heart Circ. Physiol.,
2007
Jan
, 292 (H399-407).
1241
Zheng W
et al.
Cellular distribution of the potassium channel KCNQ1 in normal mouse kidney.
Am. J. Physiol. Renal Physiol.,
2007
Jan
, 292 (F456-66).
1242
Miller TE
et al.
Whole blood RNA offers a rapid, comprehensive approach to genetic diagnosis of cardiovascular diseases.
Genet. Med.,
2007
Jan
, 9 (23-33).
1243
Jost N
et al.
Slow delayed rectifier potassium current (IKs) and the repolarization reserve.
Ann Noninvasive Electrocardiol,
2007
Jan
, 12 (64-78).
1244
Otway R
et al.
Stretch-sensitive KCNQ1 mutation A link between genetic and environmental factors in the pathogenesis of atrial fibrillation?
J. Am. Coll. Cardiol.,
2007
Feb
6
, 49 (578-86).
1245
Gao DS
et al.
QT hysteresis in long-QT syndrome children with exercise testing.
Chin. Med. J.,
2007
Feb
5
, 120 (179-82).
1246
Kwon Y
et al.
Integration of phosphoinositide- and calmodulin-mediated regulation of TRPC6.
Mol. Cell,
2007
Feb
23
, 25 (491-503).
1247
Nakajima T
et al.
HERG is protected from pharmacological block by alpha-1,2-glucosyltransferase function.
J. Biol. Chem.,
2007
Feb
23
, 282 (5506-13).
1248
Lengyel C
et al.
Diabetes mellitus attenuates the repolarization reserve in mammalian heart.
Cardiovasc. Res.,
2007
Feb
1
, 73 (512-20).
1249
Brouillette J
et al.
Characterization of ventricular repolarization in male and female guinea pigs.
J. Mol. Cell. Cardiol.,
2007
Feb
, 42 (357-66).
1250
Panaghie G
et al.
The role of S4 charges in voltage-dependent and voltage-independent KCNQ1 potassium channel complexes.
J. Gen. Physiol.,
2007
Feb
, 129 (121-33).
1251
Chevalier P
et al.
Torsades de pointes complicating atrioventricular block: evidence for a genetic predisposition.
,
2007
Feb
, 4 (170-4).
1252
Cronk LB
et al.
Novel mechanism for sudden infant death syndrome: persistent late sodium current secondary to mutations in caveolin-3.
Heart Rhythm,
2007
Feb
, 4 (161-6).
1254
Arnestad M
et al.
A mitochondrial DNA polymorphism associated with cardiac arrhythmia investigated in sudden infant death syndrome.
Acta Paediatr.,
2007
Feb
, 96 (206-10).
1255
Chen L
et al.
Mutation of an A-kinase-anchoring protein causes long-QT syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
2007
Dec
26
, 104 (20990-5).
1256
Nakamura H
et al.
Progesterone regulates cardiac repolarization through a nongenomic pathway: an in vitro patch-clamp and computational modeling study.
Circulation,
2007
Dec
18
, 116 (2913-22).
1257
Gibor G
et al.
An inactivation gate in the selectivity filter of KCNQ1 potassium channels.
Biophys. J.,
2007
Dec
15
, 93 (4159-72).
1258
Smith JA
et al.
Structural models for the KCNQ1 voltage-gated potassium channel.
Biochemistry,
2007
Dec
11
, 46 (14141-52).
1259
Boulet IR
et al.
Role of the S6 C-terminus in KCNQ1 channel gating.
J. Physiol. (Lond.),
2007
Dec
1
, 585 (325-37).
1260
Bajwa PJ
et al.
Fenofibrate inhibits intestinal Cl- secretion by blocking basolateral KCNQ1 K+ channels.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2007
Dec
, 293 (G1288-99).
1261
Sandu C
et al.
Role of the serum and glucocorticoid inducible kinase SGK1 in glucocorticoid stimulation of gastric acid secretion.
Pflugers Arch.,
2007
Dec
, 455 (493-503).
1262
Couderc JP
et al.
Impaired T-amplitude adaptation to heart rate characterizes I(Kr) inhibition in the congenital and acquired forms of the long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2007
Dec
, 18 (1299-305).
1263
Tseng GN
The phenotype of a KCNQ1 mutation depends on its KCNE partners: is the cardiac slow delayed rectifier (IKs) channel more than a KCNQ1/KCNE1 complex?
Heart Rhythm,
2007
Dec
, 4 (1542-3).
1264
Lundby A
et al.
KCNQ1 mutation Q147R is associated with atrial fibrillation and prolonged QT interval.
Heart Rhythm,
2007
Dec
, 4 (1532-41).
1265
Muñoz V
et al.
Adenoviral expression of IKs contributes to wavebreak and fibrillatory conduction in neonatal rat ventricular cardiomyocyte monolayers.
Circ. Res.,
2007
Aug
31
, 101 (475-83).
1266
O'Mahony F
et al.
Female gender-specific inhibition of KCNQ1 channels and chloride secretion by 17beta-estradiol in rat distal colonic crypts.
J. Biol. Chem.,
2007
Aug
24
, 282 (24563-73).
1267
Knollmann BC
et al.
Kcnq1 contributes to an adrenergic-sensitive steady-state K+ current in mouse heart.
Biochem. Biophys. Res. Commun.,
2007
Aug
17
, 360 (212-8).
1268
Walker VE
et al.
Co-chaperone FKBP38 promotes HERG trafficking.
J. Biol. Chem.,
2007
Aug
10
, 282 (23509-16).
1269
Hu D
et al.
Novel mutation in the SCN5A gene associated with arrhythmic storm development during acute myocardial infarction.
,
2007
Aug
, 4 (1072-80).
1270
Kim JK
et al.
Effects of sevoflurane on the cAMP-induced short-circuit current in mouse tracheal epithelium and recombinant Cl- (CFTR) and K+ (KCNQ1) channels.
,
2007
Aug
, 99 (245-51).
1271
Luo X
et al.
Transcriptional activation by stimulating protein 1 and post-transcriptional repression by muscle-specific microRNAs of IKs-encoding genes and potential implications in regional heterogeneity of their expressions.
J. Cell. Physiol.,
2007
Aug
, 212 (358-67).
1272
Raviña T
et al.
Acquired long QT syndrome: risperidone-facilitated triggered activity and Torsades de Pointes during complete AV block. I.
Int. J. Cardiol.,
2007
Apr
4
, 116 (416-20).
1273
Jensen HS
et al.
Inactivation as a new regulatory mechanism for neuronal Kv7 channels.
Biophys. J.,
2007
Apr
15
, 92 (2747-56).
1274
Jespersen T
et al.
The KCNQ1 potassium channel is down-regulated by ubiquitylating enzymes of the Nedd4/Nedd4-like family.
Cardiovasc. Res.,
2007
Apr
1
, 74 (64-74).
1275
Volders PG
et al.
Mapping a novel locus for familial atrial fibrillation on chromosome 10p11-q21.
Heart Rhythm,
2007
Apr
, 4 (469-75).
1276
Liu XS
et al.
Probing the interaction between KCNE2 and KCNQ1 in their transmembrane regions.
J. Membr. Biol.,
2007
Apr
, 216 (117-27).
1277
Watanabe E
et al.
Upregulation of KCNE1 induces QT interval prolongation in patients with chronic heart failure.
Circ. J.,
2007
Apr
, 71 (471-8).
1278
Alonso-Atienza F
et al.
Action potential alternans in LQT3 syndrome: a simulation study.
,
2007
, 2007 (640-3).
1280
Bankston JR
et al.
A novel and lethal de novo LQT-3 mutation in a newborn with distinct molecular pharmacology and therapeutic response.
PLoS ONE,
2007
, 2 (e1258).
1281
Heitzmann D
et al.
KCNE beta subunits determine pH sensitivity of KCNQ1 potassium channels.
Cell. Physiol. Biochem.,
2007
, 19 (21-32).
1282
Berecki G
et al.
Cardiac channelopathies studied with the dynamic action potential-clamp technique.
Methods Mol. Biol.,
2007
, 403 (233-50).
1283
Zeng Z
et al.
The single nucleotide polymorphisms of I(Ks) potassium channel genes and their association with atrial fibrillation in a Chinese population.
Cardiology,
2007
, 108 (97-103).
1284
Márquez MF
et al.
[KCNQ 1 (KvLQT1) missense mutation causing congenital long QT syndrome (Jervell-Lange-Nielsen) in a Mexican family]
,
2006 Jul-Sep
, 76 (257-62).
1285
Millat G
et al.
Spectrum of pathogenic mutations and associated polymorphisms in a cohort of 44 unrelated patients with long QT syndrome.
Clin. Genet.,
2006
Sep
, 70 (214-27).
1286
Novotny T
et al.
The homozygous KCNQ1 gene mutation associated with recessive Romano-Ward syndrome.
,
2006
Sep
, 29 (1013-5).
1287
Siebrands CC
et al.
Long QT 1 mutation KCNQ1A344V increases local anesthetic sensitivity of the slowly activating delayed rectifier potassium current.
Anesthesiology,
2006
Sep
, 105 (511-20).
1288
Vatta M
et al.
Mutations in KCNE1 in long QT syndrome (LQTS): insights into mechanism of LQTS and drug sensitivity?
Heart Rhythm,
2006
Sep
, 3 (1041-2).
1289
Wu DM
et al.
Characterization of an LQT5-related mutation in KCNE1, Y81C: implications for a role of KCNE1 cytoplasmic domain in IKs channel function.
Heart Rhythm,
2006
Sep
, 3 (1031-40).
1290
Heradien MJ
et al.
Does pregnancy increase cardiac risk for LQT1 patients with the KCNQ1-A341V mutation?
J. Am. Coll. Cardiol.,
2006
Oct
3
, 48 (1410-5).
1291
Choe CU
et al.
C-terminal HERG (LQT2) mutations disrupt IKr channel regulation through 14-3-3epsilon.
Hum. Mol. Genet.,
2006
Oct
1
, 15 (2888-902).
1292
Knipper M
et al.
Deafness in LIMP2-deficient mice due to early loss of the potassium channel KCNQ1/KCNE1 in marginal cells of the stria vascularis.
J. Physiol. (Lond.),
2006
Oct
1
, 576 (73-86).
1293
Stengl M
et al.
Temporal patterns of electrical remodeling in canine ventricular hypertrophy: focus on IKs downregulation and blunted beta-adrenergic activation.
Cardiovasc. Res.,
2006
Oct
1
, 72 (90-100).
1294
Zhou X
et al.
Infantile seizures and other epileptic phenotypes in a Chinese family with a missense mutation of KCNQ2.
Eur. J. Pediatr.,
2006
Oct
, 165 (691-5).
1295
Lang F
et al.
(Patho)physiological significance of the serum- and glucocorticoid-inducible kinase isoforms.
Physiol. Rev.,
2006
Oct
, 86 (1151-78).
1296
Ohmoto M
et al.
Taste receptor cells express voltage-dependent potassium channels in a cell age-specific manner.
Chem. Senses,
2006
Oct
, 31 (739-46).
1297
Perhonen MA
et al.
Effect of physical training on ventricular repolarization in type 1 long QT syndrome: a pilot study in asymptomatic carriers of the G589D KCNQ1 mutation.
,
2006
Oct
, 8 (894-8).
1298
Betge S
et al.
[Long QT syndrome causing grand mal epilepsy: case report, pedigree, therapeutic options, and review of the literature]
,
2006
Oct
, 77 (1210-7).
1300
Niu DM
et al.
A common SCN5A polymorphism attenuates a severe cardiac phenotype caused by a nonsense SCN5A mutation in a Chinese family with an inherited cardiac conduction defect.
J. Med. Genet.,
2006
Oct
, 43 (817-21).
1301
Missan S
et al.
Role of kinases and G-proteins in the hyposmotic stimulation of cardiac IKs.
Biochim. Biophys. Acta,
2006
Oct
, 1758 (1641-52).
1302
Kanters JK
et al.
Long QT syndrome genotyping by electrocardiography: fact, fiction, or something in between?
,
2006
Oct
, 39 (S119-22).
1303
Fredj S
et al.
Altered Na+ channels promote pause-induced spontaneous diastolic activity in long QT syndrome type 3 myocytes.
Circ. Res.,
2006
Nov
24
, 99 (1225-32).
1304
Zehelein J
et al.
Skipping of Exon 1 in the KCNQ1 gene causes Jervell and Lange-Nielsen syndrome.
J. Biol. Chem.,
2006
Nov
17
, 281 (35397-403).
1305
Vatta M
et al.
Mutant caveolin-3 induces persistent late sodium current and is associated with long-QT syndrome.
Circulation,
2006
Nov
14
, 114 (2104-12).
1306
Dahimène S
et al.
The N-terminal juxtamembranous domain of KCNQ1 is critical for channel surface expression: implications in the Romano-Ward LQT1 syndrome.
Circ. Res.,
2006
Nov
10
, 99 (1076-83).
1307
Bett GC
et al.
Ancillary subunits and stimulation frequency determine the potency of chromanol 293B block of the KCNQ1 potassium channel.
J. Physiol. (Lond.),
2006
Nov
1
, 576 (755-67).
1308
Limberis JT
et al.
Altering extracellular potassium concentration does not modulate drug block of human ether-a-go-go-related gene (hERG) channels.
Clin. Exp. Pharmacol. Physiol.,
2006
Nov
, 33 (1059-65).
1309
Roy J
et al.
Exposure to sodium butyrate leads to functional downregulation of calcium-activated potassium channels in human airway epithelial cells.
Pflugers Arch.,
2006
Nov
, 453 (167-76).
1310
Nakano S
et al.
Expression profile of LIT1/KCNQ1OT1 and epigenetic status at the KvDMR1 in colorectal cancers.
Cancer Sci.,
2006
Nov
, 97 (1147-54).
1311
Lewis A
et al.
Epigenetic dynamics of the Kcnq1 imprinted domain in the early embryo.
Development,
2006
Nov
, 133 (4203-10).
1312
Iwasaki YK
et al.
A method for the simultaneous analysis of mRNA levels of multiple cardiac ion channels with a multi-probe RNase protection assay.
,
2006
Nov
, 8 (1011-5).
1313
Bentzen BH
et al.
The acrylamide (S)-1 differentially affects Kv7 (KCNQ) potassium channels.
Neuropharmacology,
2006
Nov
, 51 (1068-77).
1314
Chu HQ
et al.
[Mice lacking the marginal cell KCNQ1 have impaired cochlear potassium cycling are profoundly deaf].
Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi,
2006
Nov
, 41 (867-8).
1315
Diness TG
et al.
Frequency-dependent modulation of KCNQ1 and HERG1 potassium channels.
Biochem. Biophys. Res. Commun.,
2006
May
19
, 343 (1224-33).
1316
Kanduri C
et al.
The length of the transcript encoded from the Kcnq1ot1 antisense promoter determines the degree of silencing.
EMBO J.,
2006
May
17
, 25 (2096-106).
1317
Kapetanopoulos A
et al.
The congenital long QT syndrome and implications for young athletes.
,
2006
May
, 38 (816-25).
1318
Kang J
et al.
Mechanisms underlying the QT interval-prolonging effects of sevoflurane and its interactions with other QT-prolonging drugs.
Anesthesiology,
2006
May
, 104 (1015-22).
1319
Xu HD
et al.
Parentally imprinted allele typing at a short tandem repeat locus in intron 1a of imprinted gene KCNQ1.
,
2006
May
, 8 (139-43).
1320
Restano-Cassulini R
et al.
Species diversity and peptide toxins blocking selectivity of ether-a-go-go-related gene subfamily K+ channels in the central nervous system.
Mol. Pharmacol.,
2006
May
, 69 (1673-83).
1321
Park JH
et al.
Segmental heterogeneity of electrogenic secretions in human ascending colon and rectum.
,
2006
May
, 21 (357-64).
1322
Wedekind H
et al.
Sudden infant death syndrome and long QT syndrome: an epidemiological and genetic study.
Int. J. Legal Med.,
2006
May
, 120 (129-37).
1323
Tan HL
Sodium channel variants in heart disease: expanding horizons.
J. Cardiovasc. Electrophysiol.,
2006
May
, 17 Suppl 1 (S151-S157).
1324
Ching CK
et al.
Congenital long QT syndromes: clinical features, molecular genetics and genetic testing.
Expert Rev. Mol. Diagn.,
2006
May
, 6 (365-74).
1325
Fedida D
et al.
The role of late I and antiarrhythmic drugs in EAD formation and termination in Purkinje fibers.
J. Cardiovasc. Electrophysiol.,
2006
May
, 17 Suppl 1 (S71-S78).
1326
Carmeliet E
Action potential duration, rate of stimulation, and intracellular sodium.
J. Cardiovasc. Electrophysiol.,
2006
May
, 17 Suppl 1 (S2-S7).
1327
Fredj S
et al.
Molecular basis of ranolazine block of LQT-3 mutant sodium channels: evidence for site of action.
Br. J. Pharmacol.,
2006
May
, 148 (16-24).
1328
Jespersen T
et al.
The corticosteroid hormone induced factor: a new modulator of KCNQ1 channels?
Biochem. Biophys. Res. Commun.,
2006
Mar
24
, 341 (979-88).
1329
Vyas H
et al.
Epinephrine QT stress testing in the evaluation of congenital long-QT syndrome: diagnostic accuracy of the paradoxical QT response.
Circulation,
2006
Mar
21
, 113 (1385-92).
1330
Seebohm G
et al.
Differential roles of S6 domain hinges in the gating of KCNQ potassium channels.
Biophys. J.,
2006
Mar
15
, 90 (2235-44).
1331
Huang H
et al.
Nav1.5/R1193Q polymorphism is associated with both long QT and Brugada syndromes.
,
2006
Mar
15
, 22 (309-13).
1332
Leng Q
et al.
WNK3, a kinase related to genes mutated in hereditary hypertension with hyperkalaemia, regulates the K+ channel ROMK1 (Kir1.1).
J. Physiol. (Lond.),
2006
Mar
1
, 571 (275-86).
1333
Koo SH
et al.
Genetic polymorphisms in KCNQ1, HERG, KCNE1 and KCNE2 genes in the Chinese, Malay and Indian populations of Singapore.
,
2006
Mar
, 61 (301-8).
1334
de Castro MP
et al.
Protein distribution of Kcnq1, Kcnh2, and Kcne3 potassium channel subunits during mouse embryonic development.
,
2006
Mar
, 288 (304-15).
1335
Modell SM
et al.
The long QT syndrome family of cardiac ion channelopathies: a HuGE review.
Genet. Med.,
2006
Mar
, 8 (143-55).
1336
Rasche S
et al.
[Long QT syndrome and anaesthesia]
Anaesthesist,
2006
Mar
, 55 (229-46).
1337
Tsuboi M
et al.
Cellular basis for electrocardiographic and arrhythmic manifestations of Andersen-Tawil syndrome (LQT7).
Heart Rhythm,
2006
Mar
, 3 (328-35).
1338
Vecchietti S
et al.
Computer simulation of wild-type and mutant human cardiac Na+ current.
,
2006
Mar
, 44 (35-44).
1339
Toyoda F
et al.
Modulation of functional properties of KCNQ1 channel by association of KCNE1 and KCNE2.
Biochem. Biophys. Res. Commun.,
2006
Jun
9
, 344 (814-20).
1340
Bundgaard H
et al.
[Long QT syndrome--genes, mechanisms and risks. Indication for genetic family screening?]
Ugeskr. Laeg.,
2006
Jun
26
, 168 (2537-42).
1341
Pelish HE
et al.
The Cdc42 inhibitor secramine B prevents cAMP-induced K+ conductance in intestinal epithelial cells.
Biochem. Pharmacol.,
2006
Jun
14
, 71 (1720-6).
1342
Varró A
et al.
Low penetrance, subclinical congenital LQTS: concealed LQTS or silent LQTS?
Cardiovasc. Res.,
2006
Jun
1
, 70 (404-6).
1343
Boulet IR
et al.
Functional effects of a KCNQ1 mutation associated with the long QT syndrome.
Cardiovasc. Res.,
2006
Jun
1
, 70 (466-74).
1344
Ozawa T
et al.
Gender and age effects on ventricular repolarization abnormality in Japanese general carriers of a G643S common single nucleotide polymorphism for the KCNQ1 gene.
Circ. J.,
2006
Jun
, 70 (645-50).
1345
Liu WL
et al.
[Novel mutations of potassium channel KCNQ1 S145L and KCNH2 Y475C genes in Chinese pedigrees of long QT syndrome]
Zhonghua Nei Ke Za Zhi,
2006
Jun
, 45 (463-6).
1346
She HR
et al.
[Electrophysiological characterization of long QT syndrome associated mutations V630A and N633S]
,
2006
Jun
, 34 (523-7).
1347
Shuman C
et al.
Constitutional UPD for chromosome 11p15 in individuals with isolated hemihyperplasia is associated with high tumor risk and occurs following assisted reproductive technologies.
Am. J. Med. Genet. A,
2006
Jul
15
, 140 (1497-503).
1348
Gargus JJ
Ion channel functional candidate genes in multigenic neuropsychiatric disease.
Biol. Psychiatry,
2006
Jul
15
, 60 (177-85).
1349
Obreztchikova MN
et al.
I(Kr) contributes to the altered ventricular repolarization that determines long-term cardiac memory.
Cardiovasc. Res.,
2006
Jul
1
, 71 (88-96).
1350
Lan WZ
et al.
Modulation of hepatocellular swelling-activated K+ currents by phosphoinositide pathway-dependent protein kinase C.
Am. J. Physiol., Cell Physiol.,
2006
Jul
, 291 (C93-103).
1351
Struijk JJ
et al.
Classification of the long-QT syndrome based on discriminant analysis of T-wave morphology.
,
2006
Jul
, 44 (543-9).
1352
Patel ND
et al.
The heterogeneous spectrum of the long QT syndrome.
Eur. J. Intern. Med.,
2006
Jul
, 17 (235-40).
1354
Tester DJ
et al.
Genotypic heterogeneity and phenotypic mimicry among unrelated patients referred for catecholaminergic polymorphic ventricular tachycardia genetic testing.
Heart Rhythm,
2006
Jul
, 3 (800-5).
1355
Tester DJ
et al.
Allelic dropout in long QT syndrome genetic testing: a possible mechanism underlying false-negative results.
Heart Rhythm,
2006
Jul
, 3 (815-21).
1356
Scornik FS
et al.
Functional expression of "cardiac-type" Nav1.5 sodium channel in canine intracardiac ganglia.
Heart Rhythm,
2006
Jul
, 3 (842-50).
1357
Roberts R
Genomics and cardiac arrhythmias.
J. Am. Coll. Cardiol.,
2006
Jan
3
, 47 (9-21).
1358
Tsuji Y
et al.
Potassium channel subunit remodeling in rabbits exposed to long-term bradycardia or tachycardia: discrete arrhythmogenic consequences related to differential delayed-rectifier changes.
Circulation,
2006
Jan
24
, 113 (345-55).
1359
Anderson CL
et al.
Most LQT2 mutations reduce Kv11.1 (hERG) current by a class 2 (trafficking-deficient) mechanism.
Circulation,
2006
Jan
24
, 113 (365-73).
1360
Berecki G
et al.
Long-QT syndrome-related sodium channel mutations probed by the dynamic action potential clamp technique.
J. Physiol. (Lond.),
2006
Jan
15
, 570 (237-50).
1361
Sun BW
et al.
Temporal and parental-specific expression of imprinted genes in a newly derived Chinese human embryonic stem cell line and embryoid bodies.
Hum. Mol. Genet.,
2006
Jan
1
, 15 (65-75).
1362
Beatty L
et al.
Detailed analysis of the methylation patterns of the KvDMR1 imprinting control region of human chromosome 11.
Genomics,
2006
Jan
, 87 (46-56).
1363
Rodriguez-Menchaca A
et al.
Block of HERG channels by berberine: mechanisms of voltage- and state-dependence probed with site-directed mutant channels.
J. Cardiovasc. Pharmacol.,
2006
Jan
, 47 (21-9).
1364
Schwartz PJ
The congenital long QT syndromes from genotype to phenotype: clinical implications.
J. Intern. Med.,
2006
Jan
, 259 (39-47).
1365
Rudy Y
Modelling and imaging cardiac repolarization abnormalities.
J. Intern. Med.,
2006
Jan
, 259 (91-106).
1366
Koopmann TT
et al.
Long QT syndrome caused by a large duplication in the KCNH2 (HERG) gene undetectable by current polymerase chain reaction-based exon-scanning methodologies.
Heart Rhythm,
2006
Jan
, 3 (52-5).
1367
Tester DJ
et al.
Effect of clinical phenotype on yield of long QT syndrome genetic testing.
J. Am. Coll. Cardiol.,
2006
Feb
21
, 47 (764-8).
1368
Möller C
et al.
Effects of estradiol on cardiac ion channel currents.
Eur. J. Pharmacol.,
2006
Feb
17
, 532 (44-9).
1369
Schwartz PJ
et al.
The Jervell and Lange-Nielsen syndrome: natural history, molecular basis, and clinical outcome.
Circulation,
2006
Feb
14
, 113 (783-90).
1370
Gohma H
et al.
WTC deafness Kyoto (dfk): a rat model for extensive investigations of Kcnq1 functions.
Physiol. Genomics,
2006
Feb
14
, 24 (198-206).
1371
Panaghie G
et al.
Interaction of KCNE subunits with the KCNQ1 K+ channel pore.
J. Physiol. (Lond.),
2006
Feb
1
, 570 (455-67).
1372
De Bruin ML
et al.
Pharmacogenetics of drug-induced arrhythmias: a feasibility study using spontaneous adverse drug reactions reporting data.
,
2006
Feb
, 15 (99-105).
1373
Iraqi M
et al.
[Long QT syndrome in children: analysis of the Lyon series]
Arch Mal Coeur Vaiss,
2006
Feb
, 99 (134-40).
1374
Fossa AA
et al.
QT prolongation modifies dynamic restitution and hysteresis of the beat-to-beat QT-TQ interval relationship during normal sinus rhythm under varying states of repolarization.
J. Pharmacol. Exp. Ther.,
2006
Feb
, 316 (498-506).
1375
Tian M
et al.
Effects of the antifungal antibiotic clotrimazole on human cardiac repolarization potassium currents.
Br. J. Pharmacol.,
2006
Feb
, 147 (289-97).
1376
Creighton W
et al.
Identification of novel missense mutations of cardiac ryanodine receptor gene in exercise-induced sudden death at autopsy.
,
2006
Feb
, 8 (62-7).
1377
Sarzani R
et al.
Expression of potassium channel isoforms mRNA in normal human adrenals and aldosterone-secreting adenomas.
J. Endocrinol. Invest.,
2006
Feb
, 29 (147-53).
1378
Trepakova ES
et al.
Flunarizine is a highly potent inhibitor of cardiac hERG potassium current.
J. Cardiovasc. Pharmacol.,
2006
Feb
, 47 (211-20).
1379
Novotný T
et al.
[Mutational analysis of LQT genes in individuals with drug induced QT interval prolongation]
,
2006
Feb
, 52 (116-8).
1380
Couderc JP
et al.
Repolarization morphology in adult LQT2 carriers with borderline prolonged QTc interval.
Heart Rhythm,
2006
Dec
, 3 (1460-6).
1382
Rossignol S
et al.
The epigenetic imprinting defect of patients with Beckwith-Wiedemann syndrome born after assisted reproductive technology is not restricted to the 11p15 region.
J. Med. Genet.,
2006
Dec
, 43 (902-7).
1383
Li Y
et al.
[Construction of eukaryotic expression vector of congenital long QT syndrome related HERG gene A561V mutation and its expression in HEK293 cells]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2006
Dec
, 23 (627-30).
1384
Wu DM
et al.
KCNE2 is colocalized with KCNQ1 and KCNE1 in cardiac myocytes and may function as a negative modulator of I(Ks) current amplitude in the heart.
,
2006
Dec
, 3 (1469-80).
1385
Mank-Seymour AR
et al.
Association of torsades de pointes with novel and known single nucleotide polymorphisms in long QT syndrome genes.
Am. Heart J.,
2006
Dec
, 152 (1116-22).
1386
Rocheleau JM
et al.
Secondary structure of a KCNE cytoplasmic domain.
J. Gen. Physiol.,
2006
Dec
, 128 (721-9).
1387
Leroy C
et al.
Regulation of ENaC and CFTR expression with K+ channel modulators and effect on fluid absorption across alveolar epithelial cells.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2006
Dec
, 291 (L1207-19).
1388
Roepke TK
et al.
The KCNE2 potassium channel ancillary subunit is essential for gastric acid secretion.
J. Biol. Chem.,
2006
Aug
18
, 281 (23740-7).
1389
Viitasalo M
et al.
Effects of beta-blocker therapy on ventricular repolarization documented by 24-h electrocardiography in patients with type 1 long-QT syndrome.
J. Am. Coll. Cardiol.,
2006
Aug
15
, 48 (747-53).
1390
Russo S
et al.
Molecular and genomic characterisation of cryptic chromosomal alterations leading to paternal duplication of the 11p15.5 Beckwith-Wiedemann region.
J. Med. Genet.,
2006
Aug
, 43 (e39).
1391
Van Laer L
et al.
The contribution of genes involved in potassium-recycling in the inner ear to noise-induced hearing loss.
Hum. Mutat.,
2006
Aug
, 27 (786-95).
1392
Jow F
et al.
Rb+ efflux through functional activation of cardiac KCNQ1/minK channels by the benzodiazepine R-L3 (L-364,373).
,
2006
Aug
, 4 (443-50).
1393
Schwake M
et al.
Structural determinants of M-type KCNQ (Kv7) K+ channel assembly.
J. Neurosci.,
2006
Apr
5
, 26 (3757-66).
1394
Ghosh S
et al.
KCNQ1 assembly and function is blocked by long-QT syndrome mutations that disrupt interaction with calmodulin.
Circ. Res.,
2006
Apr
28
, 98 (1048-54).
1395
Shamgar L
et al.
Calmodulin is essential for cardiac IKS channel gating and assembly: impaired function in long-QT mutations.
Circ. Res.,
2006
Apr
28
, 98 (1055-63).
1396
Monk D
et al.
Limited evolutionary conservation of imprinting in the human placenta.
Proc. Natl. Acad. Sci. U.S.A.,
2006
Apr
25
, 103 (6623-8).
1397
Teixeira M
et al.
Functional IsK/KvLQT1 potassium channel in a new corticosteroid-sensitive cell line derived from the inner ear.
J. Biol. Chem.,
2006
Apr
14
, 281 (10496-507).
1398
Liang GH
et al.
Molecular analyses of KCNQ1-5 potassium channel mRNAs in rat and guinea pig inner ears: expression, cloning, and alternative splicing.
Acta Otolaryngol.,
2006
Apr
, 126 (346-52).
1399
Harrison-Woolrych M
et al.
QT interval prolongation associated with sibutramine treatment.
,
2006
Apr
, 61 (464-9).
1400
Anastasakis A
et al.
Phenotype reveals genotype in a Greek long QT syndrome family.
,
2006
Apr
, 8 (241-4).
1401
Palmer ML
et al.
Protease-activated receptor regulation of Cl- secretion in Calu-3 cells requires prostaglandin release and CFTR activation.
Am. J. Physiol., Cell Physiol.,
2006
Apr
, 290 (C1189-98).
1403
Dong MQ
et al.
Characterization of recombinant human cardiac KCNQ1/KCNE1 channels (I (Ks)) stably expressed in HEK 293 cells.
J. Membr. Biol.,
2006
Apr
, 210 (183-92).
1404
Ellinor PT
et al.
Potassium channel gene mutations rarely cause atrial fibrillation.
BMC Med. Genet.,
2006
, 7 (70).
1405
Tester DJ
et al.
Mutation detection in congenital long QT syndrome: cardiac channel gene screen using PCR, dHPLC, and direct DNA sequencing.
Methods Mol. Med.,
2006
, 128 (181-207).
1406
Fodstad H
et al.
Molecular characterization of two founder mutations causing long QT syndrome and identification of compound heterozygous patients.
Ann. Med.,
2006
, 38 (294-304).
1407
Strutz-Seebohm N
et al.
Functional coassembly of KCNQ4 with KCNE-beta- subunits in Xenopus oocytes.
Cell. Physiol. Biochem.,
2006
, 18 (57-66).
1408
Loussouarn G
et al.
KCNQ1 K+ channel-mediated cardiac channelopathies.
Methods Mol. Biol.,
2006
, 337 (167-83).
1409
Felipe A
et al.
Potassium channels: new targets in cancer therapy.
Cancer Detect. Prev.,
2006
, 30 (375-85).
1410
Robertson GA
et al.
HERG trafficking and pharmacological rescue of LQTS-2 mutant channels.
Handb Exp Pharmacol,
2006
, (349-55).
1411
Higashimoto K
et al.
Imprinting disruption of the CDKN1C/KCNQ1OT1 domain: the molecular mechanisms causing Beckwith-Wiedemann syndrome and cancer.
Cytogenet. Genome Res.,
2006
, 113 (306-12).
1412
Wible BA
et al.
HERG-Lite: a novel comprehensive high-throughput screen for drug-induced hERG risk.
,
2005 Jul-Aug
, 52 (136-45).
1413
Guo L
et al.
Automated electrophysiology in the preclinical evaluation of drugs for potential QT prolongation.
,
2005 Jul-Aug
, 52 (123-35).
1414
Shim SH
et al.
Gene sequencing in neonates and infants with the long QT syndrome.
Genet. Test.,
2005
Winter
, 9 (281-4).
1415
Chen L
et al.
Phosphorylation of the A-kinase-anchoring protein Yotiao contributes to protein kinase A regulation of a heart potassium channel.
J. Biol. Chem.,
2005
Sep
9
, 280 (31347-52).
1416
Silva J
et al.
Subunit interaction determines IKs participation in cardiac repolarization and repolarization reserve.
Circulation,
2005
Sep
6
, 112 (1384-91).
1417
Csanády M
et al.
[The long QT syndrome from the bedside to molecular genetic laboratory. The history of the first described Hungarian family]
,
2005
Sep
25
, 146 (2011-6).
1418
Jensen HS
et al.
The KCNQ5 potassium channel from mouse: a broadly expressed M-current like potassium channel modulated by zinc, pH, and volume changes.
Brain Res. Mol. Brain Res.,
2005
Sep
13
, 139 (52-62).
1419
Kato H
et al.
Differential diagnosis between complete and partial mole by TSSC3 antibody completely correlates to DNA diagnosis.
Diagn. Mol. Pathol.,
2005
Sep
, 14 (164-9).
1420
Gómez R
et al.
Spironolactone and its main metabolite canrenoic acid block hKv1.5, Kv4.3 and Kv7.1 + minK channels.
Br. J. Pharmacol.,
2005
Sep
, 146 (146-61).
1421
Liao T
et al.
K+ channel KVLQT1 located in the basolateral membrane of distal colonic epithelium is not essential for activating Cl- secretion.
Am. J. Physiol., Cell Physiol.,
2005
Sep
, 289 (C564-75).
1422
Brink PA
et al.
Phenotypic variability and unusual clinical severity of congenital long-QT syndrome in a founder population.
Circulation,
2005
Oct
25
, 112 (2602-10).
1423
Berge KE
et al.
[DNA-based diagnostics of long QT syndrome]
Tidsskr. Nor. Laegeforen.,
2005
Oct
20
, 125 (2783-6).
1424
Zhang M
et al.
Pharmacological properties and functional role of Kslow current in mouse pancreatic beta-cells: SK channels contribute to Kslow tail current and modulate insulin secretion.
J. Gen. Physiol.,
2005
Oct
, 126 (353-63).
1425
Tester DJ
et al.
Spectrum and prevalence of cardiac ryanodine receptor (RyR2) mutations in a cohort of unrelated patients referred explicitly for long QT syndrome genetic testing.
Heart Rhythm,
2005
Oct
, 2 (1099-105).
1426
Liu K
et al.
New mechanism contributing to drug-induced arrhythmia: rescue of a misprocessed LQT3 mutant.
Circulation,
2005
Nov
22
, 112 (3239-46).
1427
Opthof T
et al.
Transmural dispersion in LQT3 and arrhythmogenesis.
Cardiovasc. Res.,
2005
Nov
1
, 68 (336-7; author reply 338-9).
1428
Gouas L
et al.
Association of KCNQ1, KCNE1, KCNH2 and SCN5A polymorphisms with QTc interval length in a healthy population.
Eur. J. Hum. Genet.,
2005
Nov
, 13 (1213-22).
1429
Grunnet M
et al.
Functional assessment of compound mutations in the KCNQ1 and KCNH2 genes associated with long QT syndrome.
,
2005
Nov
, 2 (1238-49).
1430
Fitzgerald PT
et al.
Drug-induced torsades de pointes: the evolving role of pharmacogenetics.
,
2005
Nov
, 2 (S30-7).
1431
Zeng ZY
et al.
[The association of single nucleotide polymorphism of slow delayed rectifier K+ channel genes with atrial fibrillation in Han nationality Chinese]
Zhonghua Xin Xue Guan Bing Za Zhi,
2005
Nov
, 33 (987-91).
1432
Zhang L
et al.
Electrocardiographic features in Andersen-Tawil syndrome patients with KCNJ2 mutations: characteristic T-U-wave patterns predict the KCNJ2 genotype.
Circulation,
2005
May
31
, 111 (2720-6).
1433
Schenzer A
et al.
Molecular determinants of KCNQ (Kv7) K+ channel sensitivity to the anticonvulsant retigabine.
J. Neurosci.,
2005
May
18
, 25 (5051-60).
1434
Gong Q
et al.
Degradation of trafficking-defective long QT syndrome type II mutant channels by the ubiquitin-proteasome pathway.
J. Biol. Chem.,
2005
May
13
, 280 (19419-25).
1435
Rivas A
et al.
Inner ear abnormalities in a Kcnq1 (Kvlqt1) knockout mouse: a model of Jervell and Lange-Nielsen syndrome.
Otol. Neurotol.,
2005
May
, 26 (415-24).
1436
Tester DJ
et al.
Compendium of cardiac channel mutations in 541 consecutive unrelated patients referred for long QT syndrome genetic testing.
,
2005
May
, 2 (507-17).
1437
Rose J
et al.
Molecular correlates of altered expression of potassium currents in failing rabbit myocardium.
Am. J. Physiol. Heart Circ. Physiol.,
2005
May
, 288 (H2077-87).
1438
Eckhardt LL
et al.
Protein trafficking abnormalities: a new mechanism in drug-induced long QT syndrome.
Br. J. Pharmacol.,
2005
May
, 145 (3-4).
1439
Liu T
et al.
Sex modulates the arrhythmogenic substrate in prepubertal rabbit hearts with Long QT 2.
J. Cardiovasc. Electrophysiol.,
2005
May
, 16 (516-24).
1440
Tester DJ
et al.
Pathogenesis of unexplained drowning: new insights from a molecular autopsy.
Mayo Clin. Proc.,
2005
May
, 80 (596-600).
1441
Grunnet M
et al.
hKCNE4 inhibits the hKCNQ1 potassium current without affecting the activation kinetics.
Biochem. Biophys. Res. Commun.,
2005
Mar
25
, 328 (1146-53).
1442
Lambrecht NW
et al.
Identification of the K efflux channel coupled to the gastric H-K-ATPase during acid secretion.
Physiol. Genomics,
2005
Mar
21
, 21 (81-91).
1443
Terrenoire C
et al.
Autonomic control of cardiac action potentials: role of potassium channel kinetics in response to sympathetic stimulation.
Circ. Res.,
2005
Mar
18
, 96 (e25-34).
1444
Seebohm G
et al.
Mutation of colocalized residues of the pore helix and transmembrane segments S5 and S6 disrupt deactivation and modify inactivation of KCNQ1 K+ channels.
J. Physiol. (Lond.),
2005
Mar
1
, 563 (359-68).
1445
Lan WZ
et al.
Electrophysiological and molecular identification of hepatocellular volume-activated K+ channels.
Biochim. Biophys. Acta,
2005
Mar
1
, 1668 (223-33).
1446
Szentadrassy N
et al.
Apico-basal inhomogeneity in distribution of ion channels in canine and human ventricular myocardium.
Cardiovasc. Res.,
2005
Mar
1
, 65 (851-60).
1447
Rossenbacker T
et al.
Novel mutation in the Per-Arnt-Sim domain of KCNH2 causes a malignant form of long-QT syndrome.
Circulation,
2005
Mar
1
, 111 (961-8).
1448
Persson F
et al.
Blocking characteristics of hERG, hNav1.5, and hKvLQT1/hminK after administration of the novel anti-arrhythmic compound AZD7009.
J. Cardiovasc. Electrophysiol.,
2005
Mar
, 16 (329-41).
1449
1450
Recanatini M
et al.
QT prolongation through hERG K(+) channel blockade: current knowledge and strategies for the early prediction during drug development.
Med Res Rev,
2005
Mar
, 25 (133-66).
1451
Chen T
et al.
Reduced voltage dependence of inactivation in the SCN5A sodium channel mutation delF1617.
Am. J. Physiol. Heart Circ. Physiol.,
2005
Jun
, 288 (H2666-76).
1452
Akar FG
et al.
Molecular mechanisms underlying K+ current downregulation in canine tachycardia-induced heart failure.
Am. J. Physiol. Heart Circ. Physiol.,
2005
Jun
, 288 (H2887-96).
1453
Du R
et al.
[Relationship between congenital long QT syndrome and Brugada syndrome gene mutation]
Zhongguo Yi Xue Ke Xue Yuan Xue Bao,
2005
Jun
, 27 (289-94).
1454
Nesović J
et al.
Characterization of luminal and peritubular membrane K+ selectivity in proximal tubular cells of frog kidney.
Ann. N. Y. Acad. Sci.,
2005
Jun
, 1048 (441-4).
1455
Temple J
et al.
Atrial fibrillation in KCNE1-null mice.
Circ. Res.,
2005
Jul
8
, 97 (62-9).
1456
Du LP
et al.
Characterization of binding site of closed-state KCNQ1 potassium channel by homology modeling, molecular docking, and pharmacophore identification.
Biochem. Biophys. Res. Commun.,
2005
Jul
8
, 332 (677-87).
1457
Beaufort-Krol GC
et al.
Developmental aspects of long QT syndrome type 3 and Brugada syndrome on the basis of a single SCN5A mutation in childhood.
J. Am. Coll. Cardiol.,
2005
Jul
19
, 46 (331-7).
1458
Maron BJ
LQTS and SIDS linkage: clarifying the record.
Am. J. Cardiol.,
2005
Jul
15
, 96 (323).
1459
Akhavan A
et al.
Identification of the cyclic-nucleotide-binding domain as a conserved determinant of ion-channel cell-surface localization.
J. Cell. Sci.,
2005
Jul
1
, 118 (2803-12).
1460
Shang YP
et al.
A novel splice mutation of HERG in a Chinese family with long QT syndrome.
,
2005
Jul
, 6 (626-30).
1461
Shushi L
et al.
Clinical, genetic, and electrophysiologic characteristics of a new PAS-domain HERG mutation (M124R) causing Long QT syndrome.
Ann Noninvasive Electrocardiol,
2005
Jul
, 10 (334-41).
1462
Wolpert C
et al.
Clinical characteristics and treatment of short QT syndrome.
,
2005
Jul
, 3 (611-7).
1464
Korsgaard MP
et al.
Anxiolytic effects of Maxipost (BMS-204352) and retigabine via activation of neuronal Kv7 channels.
J. Pharmacol. Exp. Ther.,
2005
Jul
, 314 (282-92).
1465
Delisle BP
et al.
Intragenic suppression of trafficking-defective KCNH2 channels associated with long QT syndrome.
Mol. Pharmacol.,
2005
Jul
, 68 (233-40).
1466
Aiba T
et al.
Cellular and ionic mechanism for drug-induced long QT syndrome and effectiveness of verapamil.
J. Am. Coll. Cardiol.,
2005
Jan
18
, 45 (300-7).
1467
Oginosawa Y
et al.
Intrinsic mechanism of the enhanced rate-dependent QT shortening in the R1623Q mutant of the LQT3 syndrome.
Cardiovasc. Res.,
2005
Jan
1
, 65 (138-47).
1468
Melnyk P
et al.
Comparison of ion channel distribution and expression in cardiomyocytes of canine pulmonary veins versus left atrium.
Cardiovasc. Res.,
2005
Jan
1
, 65 (104-16).
1469
Frank-Hansen R
et al.
Mutations in the genes KCND2 and KCND3 encoding the ion channels Kv4.2 and Kv4.3, conducting the cardiac fast transient outward current (ITO,f), are not a frequent cause of long QT syndrome.
Clin. Chim. Acta,
2005
Jan
, 351 (95-100).
1470
Vallon V
et al.
New insights into the role of serum- and glucocorticoid-inducible kinase SGK1 in the regulation of renal function and blood pressure.
Curr. Opin. Nephrol. Hypertens.,
2005
Jan
, 14 (59-66).
1471
Walker BD
et al.
Effect of change in posture and exercise on repolarization in patients with long QT syndrome with HERG channel mutations.
,
2005
Jan
, 21 (33-8).
1472
Kuryshev YA
et al.
Pentamidine-induced long QT syndrome and block of hERG trafficking.
J. Pharmacol. Exp. Ther.,
2005
Jan
, 312 (316-23).
1473
Liu WL
et al.
[Heterozygous mutation in KCNQ1 cause Jervell and Lange-Nielsen syndrome]
Zhonghua Xin Xue Guan Bing Za Zhi,
2005
Jan
, 33 (41-4).
1474
Ramakers C
et al.
Molecular and electrical characterization of the canine cardiac ventricular septum.
J. Mol. Cell. Cardiol.,
2005
Jan
, 38 (153-61).
1475
Cerrato F
et al.
The two-domain hypothesis in Beckwith-Wiedemann syndrome: autonomous imprinting of the telomeric domain of the distal chromosome 7 cluster.
Hum. Mol. Genet.,
2005
Feb
15
, 14 (503-11).
1476
Christiansen M
et al.
Mutations in the HERG K+-ion channel: a novel link between long QT syndrome and sudden infant death syndrome.
Am. J. Cardiol.,
2005
Feb
1
, 95 (433-4).
1477
Du R
et al.
[Mutation analysis of a Chinese family with inherited long QT syndrome]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2005
Feb
, 22 (68-70).
1478
Aydin A
et al.
Single nucleotide polymorphism map of five long-QT genes.
J. Mol. Med.,
2005
Feb
, 83 (159-65).
1479
Yamaguchi M
et al.
Compound heterozygosity for mutations Asp611-->Tyr in KCNQ1 and Asp609-->Gly in KCNH2 associated with severe long QT syndrome.
Clin. Sci.,
2005
Feb
, 108 (143-50).
1480
Lundquist AL
et al.
Expression of multiple KCNE genes in human heart may enable variable modulation of I(Ks).
J. Mol. Cell. Cardiol.,
2005
Feb
, 38 (277-87).
1481
Tsevi I
et al.
KCNQ1/KCNE1 channels during germ-cell differentiation in the rat: expression associated with testis pathologies.
J. Cell. Physiol.,
2005
Feb
, 202 (400-10).
1482
Vallon V
et al.
KCNQ1-dependent transport in renal and gastrointestinal epithelia.
Proc. Natl. Acad. Sci. U.S.A.,
2005
Dec
6
, 102 (17864-9).
1483
Napolitano C
et al.
Genetic testing in the long QT syndrome: development and validation of an efficient approach to genotyping in clinical practice.
JAMA,
2005
Dec
21
, 294 (2975-80).
1484
Hong K
et al.
De novo KCNQ1 mutation responsible for atrial fibrillation and short QT syndrome in utero.
Cardiovasc. Res.,
2005
Dec
1
, 68 (433-40).
1485
Jespersen T
et al.
The KCNQ1 potassium channel: from gene to physiological function.
,
2005
Dec
, 20 (408-16).
1486
Ullrich S
et al.
Effects of I(Ks) channel inhibitors in insulin-secreting INS-1 cells.
Pflugers Arch.,
2005
Dec
, 451 (428-36).
1487
Head CE
et al.
Paced electrogram fractionation analysis of arrhythmogenic tendency in DeltaKPQ Scn5a mice.
J. Cardiovasc. Electrophysiol.,
2005
Dec
, 16 (1329-40).
1488
Crotti L
et al.
KCNH2-K897T is a genetic modifier of latent congenital long-QT syndrome.
Circulation,
2005
Aug
30
, 112 (1251-8).
1489
Paulussen AD
et al.
HERG mutation predicts short QT based on channel kinetics but causes long QT by heterotetrameric trafficking deficiency.
Cardiovasc. Res.,
2005
Aug
15
, 67 (467-75).
1490
Shimizu W
The long QT syndrome: therapeutic implications of a genetic diagnosis.
Cardiovasc. Res.,
2005
Aug
15
, 67 (347-56).
1491
Bendahhou S
et al.
In vitro molecular interactions and distribution of KCNE family with KCNQ1 in the human heart.
Cardiovasc. Res.,
2005
Aug
15
, 67 (529-38).
1492
Ehrlich JR
et al.
Atrial fibrillation-associated minK38G/S polymorphism modulates delayed rectifier current and membrane localization.
Cardiovasc. Res.,
2005
Aug
15
, 67 (520-8).
1493
Smits JP
et al.
Substitution of a conserved alanine in the domain IIIS4-S5 linker of the cardiac sodium channel causes long QT syndrome.
Cardiovasc. Res.,
2005
Aug
15
, 67 (459-66).
1494
Thomas D
et al.
Dominant-negative I(Ks) suppression by KCNQ1-deltaF339 potassium channels linked to Romano-Ward syndrome.
Cardiovasc. Res.,
2005
Aug
15
, 67 (487-97).
1495
Wilson AJ
et al.
Abnormal KCNQ1 trafficking influences disease pathogenesis in hereditary long QT syndromes (LQT1).
Cardiovasc. Res.,
2005
Aug
15
, 67 (476-86).
1497
Tester DJ
et al.
Sudden infant death syndrome: how significant are the cardiac channelopathies?
Cardiovasc. Res.,
2005
Aug
15
, 67 (388-96).
1498
Ravn LS
et al.
Relation of 97T polymorphism in KCNE5 to risk of atrial fibrillation.
Am. J. Cardiol.,
2005
Aug
1
, 96 (405-7).
1499
Szabó G
et al.
Asymmetrical distribution of ion channels in canine and human left-ventricular wall: epicardium versus midmyocardium.
Pflugers Arch.,
2005
Aug
, 450 (307-16).
1500
Dun W
et al.
Diverse phenotypes of outward currents in cells that have survived in the 5-day-infarcted heart.
Am. J. Physiol. Heart Circ. Physiol.,
2005
Aug
, 289 (H667-73).
1501
Gamper N
et al.
Structural requirements for differential sensitivity of KCNQ K+ channels to modulation by Ca2+/calmodulin.
Mol. Biol. Cell,
2005
Aug
, 16 (3538-51).
1502
Park KH
et al.
Impaired KCNQ1-KCNE1 and phosphatidylinositol-4,5-bisphosphate interaction underlies the long QT syndrome.
Circ. Res.,
2005
Apr
15
, 96 (730-9).
1503
Priori SG
et al.
A novel form of short QT syndrome (SQT3) is caused by a mutation in the KCNJ2 gene.
Circ. Res.,
2005
Apr
15
, 96 (800-7).
1504
Pfeufer A
et al.
Common variants in myocardial ion channel genes modify the QT interval in the general population: results from the KORA study.
Circ. Res.,
2005
Apr
1
, 96 (693-701).
1505
Lopes CM
et al.
PIP2 hydrolysis underlies agonist-induced inhibition and regulates voltage gating of two-pore domain K+ channels.
J. Physiol. (Lond.),
2005
Apr
1
, 564 (117-29).
1506
Dilaveris PE
Molecular predictors of drug-induced prolongation of the QT interval.
Curr Med Chem Cardiovasc Hematol Agents,
2005
Apr
, 3 (105-18).
1507
Peretz A
et al.
Meclofenamic acid and diclofenac, novel templates of KCNQ2/Q3 potassium channel openers, depress cortical neuron activity and exhibit anticonvulsant properties.
Mol. Pharmacol.,
2005
Apr
, 67 (1053-66).
1508
Meyer E
et al.
Analysis of genomic variants in the KCNQ1OT1 transcript in Silver-Russell syndrome patients.
Mol. Genet. Metab.,
2005
Apr
, 84 (376-7).
1509
Wuttke TV
et al.
The new anticonvulsant retigabine favors voltage-dependent opening of the Kv7.2 (KCNQ2) channel by binding to its activation gate.
Mol. Pharmacol.,
2005
Apr
, 67 (1009-17).
1510
Hong K
et al.
Short QT syndrome and atrial fibrillation caused by mutation in KCNH2.
J. Cardiovasc. Electrophysiol.,
2005
Apr
, 16 (394-6).
1511
Aerssens J
et al.
Pharmacogenomics and acquired long QT syndrome.
Pharmacogenomics,
2005
Apr
, 6 (259-70).
1512
1513
Seebohm G
et al.
Regulation of KCNQ4 potassium channel prepulse dependence and current amplitude by SGK1 in Xenopus oocytes.
Cell. Physiol. Biochem.,
2005
, 16 (255-62).
1514
Schwartz PJ
The long QT syndrome: a clinical counterpart of hERG mutations.
Novartis Found. Symp.,
2005
, 266 (186-98; discussion 198-203).
1515
Arima T
et al.
ZAC, LIT1 (KCNQ1OT1) and p57KIP2 (CDKN1C) are in an imprinted gene network that may play a role in Beckwith-Wiedemann syndrome.
Nucleic Acids Res.,
2005
, 33 (2650-60).
1516
Shimizu W
et al.
Specific therapy based on the genotype and cellular mechanism in inherited cardiac arrhythmias. Long QT syndrome and Brugada syndrome.
Curr. Pharm. Des.,
2005
, 11 (1561-72).
1517
Chatrath R
et al.
Beta-blocker therapy failures in symptomatic probands with genotyped long-QT syndrome.
Pediatr Cardiol,
2004 Sep-Oct
, 25 (459-65).
1518
Chiang CE
Congenital and acquired long QT syndrome. Current concepts and management.
,
2004 Jul-Aug
, 12 (222-34).
1519
Dilly KW
et al.
Overexpression of beta2-adrenergic receptors cAMP-dependent protein kinase phosphorylates and modulates slow delayed rectifier potassium channels expressed in murine heart: evidence for receptor/channel co-localization.
J. Biol. Chem.,
2004
Sep
24
, 279 (40778-87).
1520
Zhang L
et al.
An intronic mutation causes long QT syndrome.
J. Am. Coll. Cardiol.,
2004
Sep
15
, 44 (1283-91).
1521
Priori SG
et al.
Association of long QT syndrome loci and cardiac events among patients treated with beta-blockers.
JAMA,
2004
Sep
15
, 292 (1341-4).
1522
Aizawa Y
et al.
Truncated KCNQ1 mutant, A178fs/105, forms hetero-multimer channel with wild-type causing a dominant-negative suppression due to trafficking defect.
FEBS Lett.,
2004
Sep
10
, 574 (145-50).
1523
Jespersen T
et al.
Basolateral localisation of KCNQ1 potassium channels in MDCK cells: molecular identification of an N-terminal targeting motif.
J. Cell. Sci.,
2004
Sep
1
, 117 (4517-26).
1524
Wang W
Renal potassium channels: recent developments.
Curr. Opin. Nephrol. Hypertens.,
2004
Sep
, 13 (549-55).
1525
Casimiro MC
et al.
Targeted point mutagenesis of mouse Kcnq1: phenotypic analysis of mice with point mutations that cause Romano-Ward syndrome in humans.
Genomics,
2004
Sep
, 84 (555-64).
1526
Thakur N
et al.
An antisense RNA regulates the bidirectional silencing property of the Kcnq1 imprinting control region.
Mol. Cell. Biol.,
2004
Sep
, 24 (7855-62).
1528
Kanters JK
et al.
T wave morphology analysis distinguishes between KvLQT1 and HERG mutations in long QT syndrome.
Heart Rhythm,
2004
Sep
, 1 (285-92).
1529
McNair WP
et al.
SCN5A mutation associated with dilated cardiomyopathy, conduction disorder, and arrhythmia.
Circulation,
2004
Oct
12
, 110 (2163-7).
1530
Choi G
et al.
Spectrum and frequency of cardiac channel defects in swimming-triggered arrhythmia syndromes.
Circulation,
2004
Oct
12
, 110 (2119-24).
1531
Finsterer J
et al.
Skeletal muscle involvement in congenital long QT syndrome.
Neurol. Sci.,
2004
Oct
, 25 (238-40).
1532
Huffaker R
et al.
Intracellular calcium cycling, early afterdepolarizations, and reentry in simulated long QT syndrome.
Heart Rhythm,
2004
Oct
, 1 (441-8).
1533
Opdal SH
et al.
The sudden infant death syndrome gene: does it exist?
Pediatrics,
2004
Oct
, 114 (e506-12).
1534
Zehelein J
et al.
Identification and characterisation of a novel KCNQ1 mutation in a family with Romano-Ward syndrome.
Biochim. Biophys. Acta,
2004
Nov
5
, 1690 (185-92).
1535
Abriel H
et al.
Molecular and clinical determinants of drug-induced long QT syndrome: an iatrogenic channelopathy.
,
2004
Nov
27
, 134 (685-94).
1536
Kurokawa J
et al.
Regulatory actions of the A-kinase anchoring protein Yotiao on a heart potassium channel downstream of PKA phosphorylation.
Proc. Natl. Acad. Sci. U.S.A.,
2004
Nov
16
, 101 (16374-8).
1537
Elso CM
et al.
Heightened susceptibility to chronic gastritis, hyperplasia and metaplasia in Kcnq1 mutant mice.
Hum. Mol. Genet.,
2004
Nov
15
, 13 (2813-21).
1538
Bilchick K
et al.
Temporal repolarization lability differences among genotyped patients with the long QT syndrome.
Am. J. Cardiol.,
2004
Nov
15
, 94 (1312-6).
1539
Chang CC
et al.
A novel SCN5A mutation manifests as a malignant form of long QT syndrome with perinatal onset of tachycardia/bradycardia.
Cardiovasc. Res.,
2004
Nov
1
, 64 (268-78).
1540
Yang Y
et al.
Identification of a KCNE2 gain-of-function mutation in patients with familial atrial fibrillation.
Am. J. Hum. Genet.,
2004
Nov
, 75 (899-905).
1541
Skinner JR
et al.
Use of the newborn screening card to define cause of death in a 12-year-old diagnosed with epilepsy.
J Paediatr Child Health,
2004
Nov
, 40 (651-3).
1542
Ackerman MJ
et al.
Spectrum and prevalence of cardiac sodium channel variants among black, white, Asian, and Hispanic individuals: implications for arrhythmogenic susceptibility and Brugada/long QT syndrome genetic testing.
Heart Rhythm,
2004
Nov
, 1 (600-7).
1543
Chugh SS
et al.
Postmortem molecular screening in unexplained sudden death.
J. Am. Coll. Cardiol.,
2004
May
5
, 43 (1625-9).
1544
Yuill KH
et al.
Potent inhibition of human cardiac potassium (HERG) channels by the anti-estrogen agent clomiphene-without QT interval prolongation.
Biochem. Biophys. Res. Commun.,
2004
May
28
, 318 (556-61).
1545
Bellocq C
et al.
Mutation in the KCNQ1 gene leading to the short QT-interval syndrome.
Circulation,
2004
May
25
, 109 (2394-7).
1546
Leroy C
et al.
Molecular identity and function in transepithelial transport of K(ATP) channels in alveolar epithelial cells.
Am. J. Physiol. Lung Cell Mol. Physiol.,
2004
May
, 286 (L1027-37).
1547
Lee JE
et al.
Effects of KCNQ1 channel blocker, 293B, on the acetylcholine-induced Cl- secretion of rat pancreatic acini.
Pancreas,
2004
May
, 28 (435-42).
1548
Chen JZ
et al.
Single nucleotide polymorphisms of the SCN5A gene in Han Chinese and their relation with Brugada syndrome.
Chin. Med. J.,
2004
May
, 117 (652-6).
1549
Graff DW
et al.
Metal particulate matter components affect gene expression and beat frequency of neonatal rat ventricular myocytes.
Environ. Health Perspect.,
2004
May
, 112 (792-8).
1550
Wang Q
et al.
The common SCN5A mutation R1193Q causes LQTS-type electrophysiological alterations of the cardiac sodium channel.
J. Med. Genet.,
2004
May
, 41 (e66).
1551
Lupoglazoff JM
et al.
Long QT syndrome in neonates: conduction disorders associated with HERG mutations and sinus bradycardia with KCNQ1 mutations.
J. Am. Coll. Cardiol.,
2004
Mar
3
, 43 (826-30).
1552
Teng S
et al.
Clinical and electrophysiological characterization of a novel mutation R863X in HERG C-terminus associated with long QT syndrome.
J. Mol. Med.,
2004
Mar
, 82 (189-96).
1553
Wedekind H
et al.
Effective long-term control of cardiac events with beta-blockers in a family with a common LQT1 mutation.
Clin. Genet.,
2004
Mar
, 65 (233-41).
1554
Tateyama M
et al.
Structural effects of an LQT-3 mutation on heart Na+ channel gating.
Biophys. J.,
2004
Mar
, 86 (1843-51).
1555
Thu-Thuy LT
et al.
Long-term follow-up of notched T waves in female patients with LQT2 (HERG) mutations.
,
2004
Mar
, 45 (243-50).
1556
Paulussen AD
et al.
Genetic variations of KCNQ1, KCNH2, SCN5A, KCNE1, and KCNE2 in drug-induced long QT syndrome patients.
J. Mol. Med.,
2004
Mar
, 82 (182-8).
1557
Melman YF
et al.
KCNE1 binds to the KCNQ1 pore to regulate potassium channel activity.
Neuron,
2004
Jun
24
, 42 (927-37).
1558
Miller TE
et al.
Recurrent third-trimester fetal loss and maternal mosaicism for long-QT syndrome.
Circulation,
2004
Jun
22
, 109 (3029-34).
1559
Thomas D
et al.
Inhibition of cardiac HERG currents by the DNA topoisomerase II inhibitor amsacrine: mode of action.
Br. J. Pharmacol.,
2004
Jun
, 142 (485-94).
1560
Di Paolo M
et al.
Postmortem molecular analysis in victims of sudden unexplained death.
,
2004
Jun
, 25 (182-4).
1561
Li P
et al.
[The mutation scanning of KCNQ1 gene for 31 long QT syndrome families]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2004
Jun
, 21 (236-9).
1562
Lian JF
et al.
[Long QT syndrome gene diagnosis by haplotype analysis]
Zhonghua Yi Xue Yi Chuan Xue Za Zhi,
2004
Jun
, 21 (272-3).
1563
Anson BD
et al.
Molecular and functional characterization of common polymorphisms in HERG (KCNH2) potassium channels.
Am. J. Physiol. Heart Circ. Physiol.,
2004
Jun
, 286 (H2434-41).
1564
Krumerman A
et al.
An LQT mutant minK alters KvLQT1 trafficking.
Am. J. Physiol., Cell Physiol.,
2004
Jun
, 286 (C1453-63).
1565
Shimizu W
et al.
Mutation site-specific differences in arrhythmic risk and sensitivity to sympathetic stimulation in the LQT1 form of congenital long QT syndrome: multicenter study in Japan.
J. Am. Coll. Cardiol.,
2004
Jul
7
, 44 (117-25).
1566
Gouas L
et al.
New KCNQ1 mutations leading to haploinsufficiency in a general population; Defective trafficking of a KvLQT1 mutant.
Cardiovasc. Res.,
2004
Jul
1
, 63 (60-8).
1567
Knollmann BC
et al.
Isoproterenol exacerbates a long QT phenotype in Kcnq1-deficient neonatal mice: possible roles for human-like Kcnq1 isoform 1 and slow delayed rectifier K+ current.
J. Pharmacol. Exp. Ther.,
2004
Jul
, 310 (311-8).
1568
Hofman-Bang J
et al.
Does KCNE5 play a role in long QT syndrome?
Clin. Chim. Acta,
2004
Jul
, 345 (49-53).
1569
Gibor G
et al.
External barium affects the gating of KCNQ1 potassium channels and produces a pore block via two discrete sites.
J. Gen. Physiol.,
2004
Jul
, 124 (83-102).
1570
Sharma D
et al.
Characterization of a KCNQ1/KVLQT1 polymorphism in Asian families with LQT2: implications for genetic testing.
J. Mol. Cell. Cardiol.,
2004
Jul
, 37 (79-89).
1571
Greenfield SA
et al.
A novel peptide modulates alpha7 nicotinic receptor responses: implications for a possible trophic-toxic mechanism within the brain.
J. Neurochem.,
2004
Jul
, 90 (325-31).
1572
Ehrlich JR
et al.
KvLQT1 modulates the distribution and biophysical properties of HERG. A novel alpha-subunit interaction between delayed rectifier currents.
J. Biol. Chem.,
2004
Jan
9
, 279 (1233-41).
1573
Drolet B
et al.
Unusual effects of a QT-prolonging drug, arsenic trioxide, on cardiac potassium currents.
Circulation,
2004
Jan
6
, 109 (26-9).
1574
Varró A
et al.
Theoretical possibilities for the development of novel antiarrhythmic drugs.
Curr. Med. Chem.,
2004
Jan
, 11 (1-11).
1575
Hibino H
et al.
Expression of an inwardly rectifying K+ channel, Kir5.1, in specific types of fibrocytes in the cochlear lateral wall suggests its functional importance in the establishment of endocochlear potential.
Eur. J. Neurosci.,
2004
Jan
, 19 (76-84).
1576
Hong K
et al.
Phenotypic characterization of a large European family with Brugada syndrome displaying a sudden unexpected death syndrome mutation in SCN5A:.
J. Cardiovasc. Electrophysiol.,
2004
Jan
, 15 (64-9).
1577
Currid A
et al.
Chloride secretion in a morphologically differentiated human colonic cell line that expresses the epithelial Na+ channel.
J. Physiol. (Lond.),
2004
Feb
15
, 555 (241-50).
1578
Tian XL
et al.
Mechanisms by which SCN5A mutation N1325S causes cardiac arrhythmias and sudden death in vivo.
Cardiovasc. Res.,
2004
Feb
1
, 61 (256-67).
1579
Herfst LJ
et al.
Trafficking and functional expression of cardiac Na+ channels.
J. Mol. Cell. Cardiol.,
2004
Feb
, 36 (185-93).
1580
Cheng JH
et al.
Two components of delayed rectifier K+ current in heart: molecular basis, functional diversity, and contribution to repolarization.
Acta Pharmacol. Sin.,
2004
Feb
, 25 (137-45).
1581
Yumoto Y
et al.
Bepridil block of recombinant human cardiac IKs current shows a time-dependent unblock.
J. Cardiovasc. Pharmacol.,
2004
Feb
, 43 (178-82).
1582
Kobori A
et al.
Additional gene variants reduce effectiveness of beta-blockers in the LQT1 form of long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2004
Feb
, 15 (190-9).
1583
Pandey RR
et al.
NF-Y regulates the antisense promoter, bidirectional silencing, and differential epigenetic marks of the Kcnq1 imprinting control region.
J. Biol. Chem.,
2004
Dec
10
, 279 (52685-93).
1584
Saucerman JJ
et al.
Proarrhythmic consequences of a KCNQ1 AKAP-binding domain mutation: computational models of whole cells and heterogeneous tissue.
Circ. Res.,
2004
Dec
10
, 95 (1216-24).
1585
Thomas D
et al.
Direct block of hERG potassium channels by the protein kinase C inhibitor bisindolylmaleimide I (GF109203X).
Cardiovasc. Res.,
2004
Dec
1
, 64 (467-76).
1586
Heitzmann D
et al.
Heteromeric KCNE2/KCNQ1 potassium channels in the luminal membrane of gastric parietal cells.
J. Physiol. (Lond.),
2004
Dec
1
, 561 (547-57).
1587
Magyar J
et al.
Divergent action potential morphologies reveal nonequilibrium properties of human cardiac Na channels.
Cardiovasc. Res.,
2004
Dec
1
, 64 (477-87).
1588
Umlauf D
et al.
Imprinting along the Kcnq1 domain on mouse chromosome 7 involves repressive histone methylation and recruitment of Polycomb group complexes.
Nat. Genet.,
2004
Dec
, 36 (1296-300).
1589
Krishnamurthy G
et al.
Developmental evolution of the delayed rectifier current IKs in canine heart appears dependent on the beta subunit minK.
,
2004
Dec
, 1 (704-11).
1590
Gage SD
et al.
KCNE3 truncation mutants reveal a bipartite modulation of KCNQ1 K+ channels.
J. Gen. Physiol.,
2004
Dec
, 124 (759-71).
1591
Gong Q
et al.
Defective assembly and trafficking of mutant HERG channels with C-terminal truncations in long QT syndrome.
J. Mol. Cell. Cardiol.,
2004
Dec
, 37 (1225-33).
1592
Sasano T
et al.
Novel C-terminus frameshift mutation, 1122fs/147, of HERG in LQT2: additional amino acids generated by frameshift cause accelerated inactivation.
J. Mol. Cell. Cardiol.,
2004
Dec
, 37 (1205-11).
1593
Sánchez-Chapula JA
et al.
Block of wild-type and inactivation-deficient human ether-a-go-go-related gene K+ channels by halofantrine.
Naunyn Schmiedebergs Arch. Pharmacol.,
2004
Dec
, 370 (484-91).
1594
Ellinor PT
et al.
Mutations in the long QT gene, KCNQ1, are an uncommon cause of atrial fibrillation.
Heart,
2004
Dec
, 90 (1487-8).
1595
Bliek J
et al.
Epigenotyping as a tool for the prediction of tumor risk and tumor type in patients with Beckwith-Wiedemann syndrome (BWS).
J. Pediatr.,
2004
Dec
, 145 (796-9).
1596
Kanki H
et al.
A structural requirement for processing the cardiac K+ channel KCNQ1.
J. Biol. Chem.,
2004
Aug
6
, 279 (33976-83).
1597
Sinico M
et al.
Giant omphalocele and "prune belly" sequence as components of the Beckwith-Wiedemann syndrome.
Am. J. Med. Genet. A,
2004
Aug
30
, 129A (198-200).
1598
Wangemann P
et al.
Loss of KCNJ10 protein expression abolishes endocochlear potential and causes deafness in Pendred syndrome mouse model.
,
2004
Aug
20
, 2 (30).
1599
Zitron E
et al.
Human cardiac inwardly rectifying current IKir2.2 is upregulated by activation of protein kinase A.
Cardiovasc. Res.,
2004
Aug
15
, 63 (520-7).
1600
Petersen CI
et al.
In vivo identification of genes that modify ether-a-go-go-related gene activity in Caenorhabditis elegans may also affect human cardiac arrhythmia.
Proc. Natl. Acad. Sci. U.S.A.,
2004
Aug
10
, 101 (11773-8).
1601
Fodstad H
et al.
Loss-of-function mutations of the K(+) channel gene KCNJ2 constitute a rare cause of long QT syndrome.
J. Mol. Cell. Cardiol.,
2004
Aug
, 37 (593-602).
1602
Du M
et al.
The KCNQ1OT1 promoter, a key regulator of genomic imprinting in human chromosome 11p15.5.
Genomics,
2004
Aug
, 84 (288-300).
1603
Hayashi K
et al.
Probucol aggravates long QT syndrome associated with a novel missense mutation M124T in the N-terminus of HERG.
Clin. Sci.,
2004
Aug
, 107 (175-82).
1604
Westenskow P
et al.
Compound mutations: a common cause of severe long-QT syndrome.
Circulation,
2004
Apr
20
, 109 (1834-41).
1605
Jiang M
et al.
KCNE2 protein is expressed in ventricles of different species, and changes in its expression contribute to electrical remodeling in diseased hearts.
Circulation,
2004
Apr
13
, 109 (1783-8).
1606
Valdivia CR
et al.
A trafficking defective, Brugada syndrome-causing SCN5A mutation rescued by drugs.
Cardiovasc. Res.,
2004
Apr
1
, 62 (53-62).
1607
Tamargo J
et al.
Pharmacology of cardiac potassium channels.
Cardiovasc. Res.,
2004
Apr
1
, 62 (9-33).
1608
Rasmussen HB
et al.
Subcellular localization of the delayed rectifier K(+) channels KCNQ1 and ERG1 in the rat heart.
Am. J. Physiol. Heart Circ. Physiol.,
2004
Apr
, 286 (H1300-9).
1609
Koren G
Electrical remodeling and arrhythmias in long-QT syndrome: lessons from genetic models in mice.
Ann. Med.,
2004
, 36 Suppl 1 (22-7).
1610
Charpentier F
et al.
Cardiac channelopathies: from men to mice.
Ann. Med.,
2004
, 36 Suppl 1 (28-34).
1611
Busjahn A
et al.
Association of the serum and glucocorticoid regulated kinase (sgk1) gene with QT interval.
Cell. Physiol. Biochem.,
2004
, 14 (135-42).
1612
Fodstad H
et al.
Four potassium channel mutations account for 73% of the genetic spectrum underlying long-QT syndrome (LQTS) and provide evidence for a strong founder effect in Finland.
Ann. Med.,
2004
, 36 Suppl 1 (53-63).
1613
Moric-Janiszewska E
et al.
Mutational screening of SCN5A linked disorders in Polish patients and their family members.
J. Appl. Genet.,
2004
, 45 (383-90).
1614
Finley MR
et al.
Structural and functional basis for the long QT syndrome: relevance to veterinary patients.
J. Vet. Intern. Med.,
2003 Jul-Aug
, 17 (473-88).
1615
Miura M
et al.
Congenital long QT syndrome and 2:1 atrioventricular block with a mutation of the SCN5A gene.
Pediatr Cardiol,
2003 Jan-Feb
, 24 (70-2).
1616
Wang LX
Role of left cardiac sympathetic denervation in the management of congenital long QT syndrome.
,
2003 Apr-Jun
, 49 (179-81).
1617
Paulussen A
et al.
Mutation analysis in congenital Long QT Syndrome--a case with missense mutations in KCNQ1 and SCN5A.
Genet. Test.,
2003
Spring
, 7 (57-61).
1618
Wang L
Left cardiac sympathectomy prevents exercise-induced QTc prolongation in congenital long QT syndrome.
,
2003
Spring
, 8 (31-2).
1619
Chen H
et al.
Charybdotoxin binding in the I(Ks) pore demonstrates two MinK subunits in each channel complex.
Neuron,
2003
Sep
25
, 40 (15-23).
1620
Delisle BP
et al.
Thapsigargin selectively rescues the trafficking defective LQT2 channels G601S and F805C.
J. Biol. Chem.,
2003
Sep
12
, 278 (35749-54).
1621
Paavonen KJ
et al.
Functional characterization of the common amino acid 897 polymorphism of the cardiac potassium channel KCNH2 (HERG).
Cardiovasc. Res.,
2003
Sep
1
, 59 (603-11).
1622
Ma L
et al.
Characterization of a novel Long QT syndrome mutation G52R-KCNE1 in a Chinese family.
Cardiovasc. Res.,
2003
Sep
1
, 59 (612-9).
1623
Wang GK
et al.
State-dependent block of wild-type and inactivation-deficient Na+ channels by flecainide.
J. Gen. Physiol.,
2003
Sep
, 122 (365-74).
1624
Ljungstrom T
et al.
Functional coupling between heterologously expressed dopamine D(2) receptors and KCNQ channels.
Pflugers Arch.,
2003
Sep
, 446 (684-94).
1625
Liu W
et al.
Mutation analysis of potassium channel genes KCNQ1 and KCNH2 in patients with long QT syndrome.
Chin. Med. J.,
2003
Sep
, 116 (1333-5).
1626
Borlak J
et al.
Hallmarks of ion channel gene expression in end-stage heart failure.
FASEB J.,
2003
Sep
, 17 (1592-608).
1627
Cuthbert AW
et al.
Mechanisms of anion secretion in Calu-3 human airway epithelial cells by 7,8-benzoquinoline.
Br. J. Pharmacol.,
2003
Sep
, 140 (81-90).
1628
Semrád B
[Failure of antiarrhythmia agents and understanding the phenomenon of proarrhythmia]
,
2003
Sep
, 49 (700-6).
1629
Mazzadi AN
et al.
Cardiac retention of [11C]HED in genotyped long QT patients: a potential amplifier role for severity of the disease.
Am. J. Physiol. Heart Circ. Physiol.,
2003
Sep
, 285 (H1286-93).
1630
Tosaka T
et al.
Nicotine induces a long QT phenotype in Kcnq1-deficient mouse hearts.
J. Pharmacol. Exp. Ther.,
2003
Sep
, 306 (980-7).
1631
Witzenbichler B
et al.
[18-year old patient with anti-epileptic therapy and sudden cardiac death]
Z Kardiol,
2003
Sep
, 92 (747-53).
1632
Akar FG
et al.
Transmural electrophysiological heterogeneities underlying arrhythmogenesis in heart failure.
Circ. Res.,
2003
Oct
3
, 93 (638-45).
1633
Loussouarn G
et al.
Phosphatidylinositol-4,5-bisphosphate, PIP2, controls KCNQ1/KCNE1 voltage-gated potassium channels: a functional homology between voltage-gated and inward rectifier K+ channels.
EMBO J.,
2003
Oct
15
, 22 (5412-21).
1634
Seebohm G
et al.
Tight coupling of rubidium conductance and inactivation in human KCNQ1 potassium channels.
J. Physiol. (Lond.),
2003
Oct
15
, 552 (369-78).
1635
Balasubramaniam R
et al.
Electrogram prolongation and nifedipine-suppressible ventricular arrhythmias in mice following targeted disruption of KCNE1.
J. Physiol. (Lond.),
2003
Oct
15
, 552 (535-46).
1636
Akhavan A
et al.
Identification of a COOH-terminal segment involved in maturation and stability of human ether-a-go-go-related gene potassium channels.
J. Biol. Chem.,
2003
Oct
10
, 278 (40105-12).
1637
Liang L
et al.
[A novel KCNQ1 mutation in Chinese with congenital long QT syndrome]
Zhonghua Er Ke Za Zhi,
2003
Oct
, 41 (724-7).
1638
Zicha S
et al.
Molecular basis of species-specific expression of repolarizing K+ currents in the heart.
Am. J. Physiol. Heart Circ. Physiol.,
2003
Oct
, 285 (H1641-9).
1639
Beery TA
et al.
A candidate locus approach identifies a long QT syndrome gene mutation.
,
2003
Oct
, 5 (97-104).
1640
Moric E
et al.
The implications of genetic mutations in the sodium channel gene (SCN5A).
,
2003
Oct
, 5 (325-34).
1641
Kirchhof P
et al.
Prolonged atrial action potential durations and polymorphic atrial tachyarrhythmias in patients with long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2003
Oct
, 14 (1027-33).
1642
Etheridge SP
et al.
A new oral therapy for long QT syndrome: long-term oral potassium improves repolarization in patients with HERG mutations.
J. Am. Coll. Cardiol.,
2003
Nov
19
, 42 (1777-82).
1643
Seebohm G
et al.
Pharmacological activation of normal and arrhythmia-associated mutant KCNQ1 potassium channels.
Circ. Res.,
2003
Nov
14
, 93 (941-7).
1644
Thomas D
et al.
Defective protein trafficking in hERG-associated hereditary long QT syndrome (LQT2): molecular mechanisms and restoration of intracellular protein processing.
Cardiovasc. Res.,
2003
Nov
1
, 60 (235-41).
1645
Bernard K
et al.
Modulation of calcium-dependent chloride secretion by basolateral SK4-like channels in a human bronchial cell line.
J. Membr. Biol.,
2003
Nov
1
, 196 (15-31).
1646
Diaz-Meyer N
et al.
Silencing of CDKN1C (p57KIP2) is associated with hypomethylation at KvDMR1 in Beckwith-Wiedemann syndrome.
J. Med. Genet.,
2003
Nov
, 40 (797-801).
1647
Scholz EP
et al.
Drug binding to aromatic residues in the HERG channel pore cavity as possible explanation for acquired Long QT syndrome by antiparkinsonian drug budipine.
Naunyn Schmiedebergs Arch. Pharmacol.,
2003
Nov
, 368 (404-14).
1648
Thomas D
et al.
Acute effects of dronedarone on both components of the cardiac delayed rectifier K+ current, HERG and KvLQT1/minK potassium channels.
Br. J. Pharmacol.,
2003
Nov
, 140 (996-1002).
1649
Zareba W
et al.
Location of mutation in the KCNQ1 and phenotypic presentation of long QT syndrome.
J. Cardiovasc. Electrophysiol.,
2003
Nov
, 14 (1149-53).
1650
Priori SG
et al.
Risk stratification in the long-QT syndrome.
N. Engl. J. Med.,
2003
May
8
, 348 (1866-74).
1651
Clancy CE
et al.
Non-equilibrium gating in cardiac Na+ channels: an original mechanism of arrhythmia.
Circulation,
2003
May
6
, 107 (2233-7).
1652
Ohya S
et al.
Molecular variants of KCNQ channels expressed in murine portal vein myocytes: a role in delayed rectifier current.
Circ. Res.,
2003
May
16
, 92 (1016-23).
1653
Glatter KA
et al.
Tachy- or bradyarrhythmias: implications for therapeutic intervention in LQT3 families.
Circ. Res.,
2003
May
16
, 92 (941-3).
1654
Veldkamp MW
et al.
Contribution of sodium channel mutations to bradycardia and sinus node dysfunction in LQT3 families.
Circ. Res.,
2003
May
16
, 92 (976-83).
1655
Waldegger S
Heartburn: cardiac potassium channels involved in parietal cell acid secretion.
Pflugers Arch.,
2003
May
, 446 (143-7).
1656
Wehrens XH
et al.
A novel mutation L619F in the cardiac Na+ channel SCN5A associated with long-QT syndrome (LQT3): a role for the I-II linker in inactivation gating.
Hum. Mutat.,
2003
May
, 21 (552).
1658
Groenewegen WA
et al.
A novel LQT3 mutation implicates the human cardiac sodium channel domain IVS6 in inactivation kinetics.
Cardiovasc. Res.,
2003
Mar
15
, 57 (1072-8).
1659
Fabritz L
et al.
Effect of pacing and mexiletine on dispersion of repolarisation and arrhythmias in DeltaKPQ SCN5A (long QT3) mice.
Cardiovasc. Res.,
2003
Mar
15
, 57 (1085-93).
1660
Thakur N
et al.
Bidirectional silencing and DNA methylation-sensitive methylation-spreading properties of the Kcnq1 imprinting control region map to the same regions.
J. Biol. Chem.,
2003
Mar
14
, 278 (9514-9).
1661
Teng GQ
et al.
[K(+)](o)-dependent change in conformation of the HERG1 long QT mutation N629D channel results in partial reversal of the in vitro disease phenotype.
Cardiovasc. Res.,
2003
Mar
, 57 (642-50).
1662
Bosch RF
et al.
Effects of the chromanol HMR 1556 on potassium currents in atrial myocytes.
Naunyn Schmiedebergs Arch. Pharmacol.,
2003
Mar
, 367 (281-8).
1663
Ficker E
et al.
Role of the cytosolic chaperones Hsp70 and Hsp90 in maturation of the cardiac potassium channel HERG.
Circ. Res.,
2003
Jun
27
, 92 (e87-100).
1664
Burton DY
et al.
The incorporation of an ion channel gene mutation associated with the long QT syndrome (Q9E-hMiRP1) in a plasmid vector for site-specific arrhythmia gene therapy: in vitro and in vivo feasibility studies.
Hum. Gene Ther.,
2003
Jun
10
, 14 (907-22).
1665
Grunnet M
et al.
KCNQ1 channels sense small changes in cell volume.
J. Physiol. (Lond.),
2003
Jun
1
, 549 (419-27).
1666
Inoue M
et al.
Q-T peak dispersion in congenital long QT syndrome: possible marker of mutation of HERG.
Circ. J.,
2003
Jun
, 67 (495-8).
1667
Chen H
et al.
Pore- and state-dependent cadmium block of I(Ks) channels formed with MinK-55C and wild-type KCNQ1 subunits.
Biophys. J.,
2003
Jun
, 84 (3679-89).
1668
Thomas D
et al.
The antipsychotic drug chlorpromazine inhibits HERG potassium channels.
Br. J. Pharmacol.,
2003
Jun
, 139 (567-74).
1669
Zareba W
et al.
Modulating effects of age and gender on the clinical course of long QT syndrome by genotype.
J. Am. Coll. Cardiol.,
2003
Jul
2
, 42 (103-9).
1670
Yang T
et al.
Phosphorylation of the IKs channel complex inhibits drug block: novel mechanism underlying variable antiarrhythmic drug actions.
Circulation,
2003
Jul
15
, 108 (132-4).
1671
Xiao GQ
et al.
PKC isozyme selective regulation of cloned human cardiac delayed slow rectifier K current.
Biochem. Biophys. Res. Commun.,
2003
Jul
11
, 306 (1019-25).
1672
Tateyama M
et al.
Stimulation of protein kinase C inhibits bursting in disease-linked mutant human cardiac sodium channels.
Circulation,
2003
Jul
1
, 107 (3216-22).
1673
Brunner M
et al.
In vivo gene transfer of Kv1.5 normalizes action potential duration and shortens QT interval in mice with long QT phenotype.
Am. J. Physiol. Heart Circ. Physiol.,
2003
Jul
, 285 (H194-203).
1674
Seebohm G
et al.
Molecular determinants of KCNQ1 channel block by a benzodiazepine.
Mol. Pharmacol.,
2003
Jul
, 64 (70-7).
1675
Ning L
et al.
Novel compound heterozygous mutations in the KCNQ1 gene associated with autosomal recessive long QT syndrome (Jervell and Lange-Nielsen syndrome).
Ann Noninvasive Electrocardiol,
2003
Jul
, 8 (246-50).
1676
Thomas D
et al.
Inhibition of cloned HERG potassium channels by the antiestrogen tamoxifen.
Naunyn Schmiedebergs Arch. Pharmacol.,
2003
Jul
, 368 (41-8).
1677
Liu CJ
et al.
Modulation of the cardiac sodium channel Nav1.5 by fibroblast growth factor homologous factor 1B.
J. Biol. Chem.,
2003
Jan
10
, 278 (1029-36).
1678
Chen YH
et al.
KCNQ1 gain-of-function mutation in familial atrial fibrillation.
Science,
2003
Jan
10
, 299 (251-4).
1679
Kang J
et al.
Interactions of the narcotic l-alpha-acetylmethadol with human cardiac K+ channels.
Eur. J. Pharmacol.,
2003
Jan
1
, 458 (25-9).
1680
Gamper N
et al.
Subunit-specific modulation of KCNQ potassium channels by Src tyrosine kinase.
J. Neurosci.,
2003
Jan
1
, 23 (84-95).
1682
Tristani-Firouzi M
et al.
Structural determinants and biophysical properties of HERG and KCNQ1 channel gating.
J. Mol. Cell. Cardiol.,
2003
Jan
, 35 (27-35).
1683
Schwake M
et al.
A carboxy-terminal domain determines the subunit specificity of KCNQ K+ channel assembly.
EMBO Rep.,
2003
Jan
, 4 (76-81).
1684
Ma ML
et al.
Different gene expression of potassium channels by thyroid hormone and an antithyroid drug between the atrium and ventricle of rats.
,
2003
Jan
, 44 (101-10).
1685
Walker BD
et al.
Congenital and acquired long QT syndromes.
Can J Cardiol,
2003
Jan
, 19 (76-87).
1686
Le Bouter S
et al.
Microarray analysis reveals complex remodeling of cardiac ion channel expression with altered thyroid status: relation to cellular and integrated electrophysiology.
Circ. Res.,
2003
Feb
7
, 92 (234-42).
1687
Ye B
et al.
A common human SCN5A polymorphism modifies expression of an arrhythmia causing mutation.
Physiol. Genomics,
2003
Feb
6
, 12 (187-93).
1688
Ishizaki T
et al.
Loss of Igf2 imprinting in monoclonal mouse hepatic tumor cells is not associated with abnormal methylation patterns for the H19, Igf2, and Kvlqt1 differentially methylated regions.
J. Biol. Chem.,
2003
Feb
21
, 278 (6222-8).
1689
Kurokawa J
et al.
Requirement of subunit expression for cAMP-mediated regulation of a heart potassium channel.
Proc. Natl. Acad. Sci. U.S.A.,
2003
Feb
18
, 100 (2122-7).
1690
Mancini-Dinardo D
et al.
A differentially methylated region within the gene Kcnq1 functions as an imprinted promoter and silencer.
Hum. Mol. Genet.,
2003
Feb
1
, 12 (283-94).
1691
Ramakers C
et al.
Coordinated down-regulation of KCNQ1 and KCNE1 expression contributes to reduction of I(Ks) in canine hypertrophied hearts.
Cardiovasc. Res.,
2003
Feb
, 57 (486-96).
1692
Andersen PS
et al.
High-throughput single strand conformation polymorphism mutation detection by automated capillary array electrophoresis: validation of the method.
Hum. Mutat.,
2003
Feb
, 21 (116-22).
1693
Moreno I
et al.
Effects of irbesartan on cloned potassium channels involved in human cardiac repolarization.
J. Pharmacol. Exp. Ther.,
2003
Feb
, 304 (862-73).
1694
Köttgen M
et al.
P2Y6 receptor mediates colonic NaCl secretion via differential activation of cAMP-mediated transport.
J. Clin. Invest.,
2003
Feb
, 111 (371-9).
1695
Gullo F
et al.
ERG K+ channel blockade enhances firing and epinephrine secretion in rat chromaffin cells: the missing link to LQT2-related sudden death?
FASEB J.,
2003
Feb
, 17 (330-2).
1696
Embark HM
et al.
Regulation of KCNE1-dependent K(+) current by the serum and glucocorticoid-inducible kinase (SGK) isoforms.
Pflugers Arch.,
2003
Feb
, 445 (601-6).
1697
Deschênes D
et al.
Biophysical characteristics of a new mutation on the KCNQ1 potassium channel (L251P) causing long QT syndrome.
Can. J. Physiol. Pharmacol.,
2003
Feb
, 81 (129-34).
1698
Ning L
et al.
Denaturing high-performance liquid chromatography quickly and reliably detects cardiac ion channel mutations in long QT syndrome.
Genet. Test.,
2003
Fall
, 7 (249-53).
1699
Gribkoff VK
The therapeutic potential of neuronal KCNQ channel modulators.
Expert Opin. Ther. Targets,
2003
Dec
, 7 (737-48).
1700
Ackerman MJ
et al.
Ethnic differences in cardiac potassium channel variants: implications for genetic susceptibility to sudden cardiac death and genetic testing for congenital long QT syndrome.
Mayo Clin. Proc.,
2003
Dec
, 78 (1479-87).
1701
Keller DI
et al.
A novel mutation in SCN5A, delQKP 1507-1509, causing long QT syndrome: role of Q1507 residue in sodium channel inactivation.
J. Mol. Cell. Cardiol.,
2003
Dec
, 35 (1513-21).
1702
Orikabe M
et al.
Block of recombinant KCNQ1/KCNE1 K+ channels (IKs) by intracellular Na+ and its implications on action potential repolarization.
Jpn. J. Physiol.,
2003
Dec
, 53 (417-25).
1703
Du M
et al.
Insulator and silencer sequences in the imprinted region of human chromosome 11p15.5.
Hum. Mol. Genet.,
2003
Aug
1
, 12 (1927-39).
1704
Kathöfer S
et al.
Human beta(3)-adrenoreceptors couple to KvLQT1/MinK potassium channels in Xenopus oocytes via protein kinase C phosphorylation of the KvLQT1 protein.
Naunyn Schmiedebergs Arch. Pharmacol.,
2003
Aug
, 368 (119-26).
1705
Anantharam A
et al.
RNA interference reveals that endogenous Xenopus MinK-related peptides govern mammalian K+ channel function in oocyte expression studies.
J. Biol. Chem.,
2003
Apr
4
, 278 (11739-45).
1706
Maljevic S
et al.
C-terminal interaction of KCNQ2 and KCNQ3 K+ channels.
J. Physiol. (Lond.),
2003
Apr
15
, 548 (353-60).
1707
Teng S
et al.
Novel gene hKCNE4 slows the activation of the KCNQ1 channel.
Biochem. Biophys. Res. Commun.,
2003
Apr
11
, 303 (808-13).
1708
Wang L
Congenital long QT syndrome: 50 years of electrophysiological research from cell to bedside.
Acta Cardiol,
2003
Apr
, 58 (133-8).
1709
Mall M
et al.
Modulation of Ca2+-activated Cl- secretion by basolateral K+ channels in human normal and cystic fibrosis airway epithelia.
Pediatr. Res.,
2003
Apr
, 53 (608-18).
1710
Yamaguchi M
et al.
Clinical and electrophysiological characterization of a novel mutation (F193L) in the KCNQ1 gene associated with long QT syndrome.
Clin. Sci.,
2003
Apr
, 104 (377-82).
1711
Wang HW
et al.
Effect of mexiletine on long QT syndrome model.
Acta Pharmacol. Sin.,
2003
Apr
, 24 (316-20).
1712
Napolitano C
et al.
Cardiac sodium channel diseases.
Clin. Chem. Lab. Med.,
2003
Apr
, 41 (439-44).
1713
Belfodil R
et al.
CFTR-dependent and -independent swelling-activated K+ currents in primary cultures of mouse nephron.
Am. J. Physiol. Renal Physiol.,
2003
Apr
, 284 (F812-28).
1714
Chen S
et al.
KCNQ1 mutations in patients with a family history of lethal cardiac arrhythmias and sudden death.
Clin. Genet.,
2003
Apr
, 63 (273-82).
1715
Lang F
et al.
Regulation of channels by the serum and glucocorticoid-inducible kinase - implications for transport, excitability and cell proliferation.
Cell. Physiol. Biochem.,
2003
, 13 (41-50).
1716
Lai LP
et al.
Molecular genetic studies in atrial fibrillation.
Cardiology,
2003
, 100 (109-13).
1717
Liu H
et al.
Mutations in cardiac sodium channels: clinical implications.
,
2003
, 3 (173-9).
1718
Kyrmizakis DE
et al.
QT prolongation and torsades de pointes associated with concurrent use of cisapride and erythromycin.
Am J Otolaryngol,
2002 Sep-Oct
, 23 (303-7).
1719
Schutte D
et al.
Dangerous and life-threatening drugs - practical lessons from the long QT syndrome.
,
2002 Mar-Apr
, 13 (54-61).
1720
Wangemann P
K(+) cycling and its regulation in the cochlea and the vestibular labyrinth.
Audiol. Neurootol.,
2002 Jul-Aug
, 7 (199-205).
1721
Rivolta I
et al.
A novel SCN5A mutation associated with long QT-3: altered inactivation kinetics and channel dysfunction.
Physiol. Genomics,
2002
Sep
3
, 10 (191-7).
1722
Makita N
et al.
Drug-induced long-QT syndrome associated with a subclinical SCN5A mutation.
Circulation,
2002
Sep
3
, 106 (1269-74).
1723
Schreiber R
et al.
Electrolyte transport in the mouse trachea: no evidence for a contribution of luminal K(+) conductance.
J. Membr. Biol.,
2002
Sep
15
, 189 (143-51).
1724
Chen T
et al.
Enhancement of closed-state inactivation in long QT syndrome sodium channel mutation DeltaKPQ.
Am. J. Physiol. Heart Circ. Physiol.,
2002
Sep
, 283 (H966-75).
1725
Murray A
et al.
Mutation in KCNQ1 that has both recessive and dominant characteristics.
J. Med. Genet.,
2002
Sep
, 39 (681-5).
1726
Thévenod F
Ion channels in secretory granules of the pancreas and their role in exocytosis and release of secretory proteins.
Am. J. Physiol., Cell Physiol.,
2002
Sep
, 283 (C651-72).
1727
Moss AJ
T-wave patterns associated with the hereditary long QT syndrome.
,
2002
Sep
, 6 (311-5).
1728
Saab CY
et al.
Molecular determinant of Na(v)1.8 sodium channel resistance to the venom from the scorpion Leiurus quinquestriatus hebraeus.
Neurosci. Lett.,
2002
Oct
11
, 331 (79-82).
1729
Kupershmidt S
et al.
Cardiac-enriched LIM domain protein fhl2 is required to generate I(Ks) in a heterologous system.
Cardiovasc. Res.,
2002
Oct
, 56 (93-103).
1730
Doolan GK
et al.
Fatty acid augmentation of the cardiac slowly activating delayed rectifier current (IKs) is conferred by hminK.
FASEB J.,
2002
Oct
, 16 (1662-4).
1731
Gardner JD
et al.
Cisapride inhibits meal-stimulated gastric acid secretion and post-prandial gastric acidity in subjects with gastro-oesophageal reflux disease.
Aliment. Pharmacol. Ther.,
2002
Oct
, 16 (1819-29).
1732
Angelo K
et al.
KCNE5 induces time- and voltage-dependent modulation of the KCNQ1 current.
Biophys. J.,
2002
Oct
, 83 (1997-2006).
1733
Herbert E
et al.
KCNQ1 gene mutations and the respective genotype-phenotype correlations in the long QT syndrome.
Med. Sci. Monit.,
2002
Oct
, 8 (RA240-8).
1734
Neyroud N
et al.
Somatic gene transfer of tagged K+ channel fragments to probe trafficking and electrical function in epithelial cells and cardiac myocytes.
J. Membr. Biol.,
2002
Nov
15
, 190 (133-44).
1735
Han W
et al.
Comparison of ion-channel subunit expression in canine cardiac Purkinje fibers and ventricular muscle.
Circ. Res.,
2002
Nov
1
, 91 (790-7).
1736
Clancy CE
et al.
Insights into the molecular mechanisms of bradycardia-triggered arrhythmias in long QT-3 syndrome.
J. Clin. Invest.,
2002
Nov
, 110 (1251-62).
1737
Scherer CR
et al.
The antihistamine fexofenadine does not affect I(Kr) currents in a case report of drug-induced cardiac arrhythmia.
Br. J. Pharmacol.,
2002
Nov
, 137 (892-900).
1738
Bertaso F
et al.
Expression of voltage-gated K+ channels in human atrium.
Basic Res. Cardiol.,
2002
Nov
, 97 (424-33).
1739
Jongbloed R
et al.
DHPLC analysis of potassium ion channel genes in congenital long QT syndrome.
Hum. Mutat.,
2002
Nov
, 20 (382-91).
1740
Gima K
et al.
Ionic current basis of electrocardiographic waveforms: a model study.
Circ. Res.,
2002
May
3
, 90 (889-96).
1741
Kanduri C
et al.
A differentially methylated imprinting control region within the Kcnq1 locus harbors a methylation-sensitive chromatin insulator.
J. Biol. Chem.,
2002
May
17
, 277 (18106-10).
1742
Lupoglazoff JM
et al.
[Homozygotous mutation of the SCN5A gene responsible for congenital long QT syndrome with 2/1 atrioventricular block]
Arch Mal Coeur Vaiss,
2002
May
, 95 (440-6).
1743
Melman YF
et al.
KCNE regulation of KvLQT1 channels: structure-function correlates.
Trends Cardiovasc. Med.,
2002
May
, 12 (182-7).
1744
Mönnig G
et al.
[Clinical aspects and molecular genetics of the Jervell- and Lange-Nielsen Syndrome]
,
2002
May
, 91 (380-8).
1745
Blint E
et al.
Induction of p57(KIP2) expression by p73beta.
Proc. Natl. Acad. Sci. U.S.A.,
2002
Mar
19
, 99 (3529-34).
1746
Kerst G
et al.
The oxidant thimerosal modulates gating behavior of KCNQ1 by interaction with the channel outer shell.
J. Membr. Biol.,
2002
Mar
15
, 186 (89-100).
1747
Akar FG
et al.
Unique topographical distribution of M cells underlies reentrant mechanism of torsade de pointes in the long-QT syndrome.
Circulation,
2002
Mar
12
, 105 (1247-53).
1748
Clancy CE
et al.
Na(+) channel mutation that causes both Brugada and long-QT syndrome phenotypes: a simulation study of mechanism.
Circulation,
2002
Mar
12
, 105 (1208-13).
1749
Warth R
et al.
The multifaceted phenotype of the knockout mouse for the KCNE1 potassium channel gene.
Am. J. Physiol. Regul. Integr. Comp. Physiol.,
2002
Mar
, 282 (R639-48).
1750
Ko WH
et al.
Stimulation of chloride secretion by baicalein in isolated rat distal colon.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2002
Mar
, 282 (G508-18).
1751
Abbott GW
et al.
Disease-associated mutations in KCNE potassium channel subunits (MiRPs) reveal promiscuous disruption of multiple currents and conservation of mechanism.
FASEB J.,
2002
Mar
, 16 (390-400).
1753
Warth R
et al.
The role of KCNQ1/KCNE1 K(+) channels in intestine and pancreas: lessons from the KCNE1 knockout mouse.
Pflugers Arch.,
2002
Mar
, 443 (822-8).
1754
Yamada Y
et al.
A truncated splice variant of KCNQ1 cloned from rat heart.
Biochem. Biophys. Res. Commun.,
2002
Jun
7
, 294 (199-204).
1755
Rajamani S
et al.
Pharmacological rescue of human K(+) channel long-QT2 mutations: human ether-a-go-go-related gene rescue without block.
Circulation,
2002
Jun
18
, 105 (2830-5).
1756
Attali B
Human congenital long QT syndrome: more than previously thought?
Trends Pharmacol. Sci.,
2002
Jun
, 23 (249-51).
1757
Nagatomo T
et al.
Rate-dependent QT shortening mechanism for the LQT3 deltaKPQ mutant.
Cardiovasc. Res.,
2002
Jun
, 54 (624-9).
1758
Kupershmidt S
et al.
Defective human Ether-à-go-go-related gene trafficking linked to an endoplasmic reticulum retention signal in the C terminus.
J. Biol. Chem.,
2002
Jul
26
, 277 (27442-8).
1759
Drici MD
et al.
Mice display sex differences in halothane-induced polymorphic ventricular tachycardia.
Circulation,
2002
Jul
23
, 106 (497-503).
1760
Melman YF
et al.
A single transmembrane site in the KCNE-encoded proteins controls the specificity of KvLQT1 channel gating.
J. Biol. Chem.,
2002
Jul
12
, 277 (25187-94).
1761
Grunnet M
et al.
KCNE4 is an inhibitory subunit to the KCNQ1 channel.
J. Physiol. (Lond.),
2002
Jul
1
, 542 (119-30).
1762
Liu H
et al.
Channel openings are necessary but not sufficient for use-dependent block of cardiac Na(+) channels by flecainide: evidence from the analysis of disease-linked mutations.
J. Gen. Physiol.,
2002
Jul
, 120 (39-51).
1763
Finley MR
et al.
Expression and coassociation of ERG1, KCNQ1, and KCNE1 potassium channel proteins in horse heart.
Am. J. Physiol. Heart Circ. Physiol.,
2002
Jul
, 283 (H126-38).
1764
Cerrato F
et al.
The 5' end of the KCNQ1OT1 gene is hypomethylated in the Beckwith-Wiedemann syndrome.
Hum. Genet.,
2002
Jul
, 111 (105-7).
1765
Chen X
et al.
Inhibitory effects of volatile anesthetics on currents produced on heterologous expression of KvLQT1 and minK in Xenopus oocytes.
Vascul. Pharmacol.,
2002
Jul
, 39 (33-8).
1766
Marx SO
et al.
Requirement of a macromolecular signaling complex for beta adrenergic receptor modulation of the KCNQ1-KCNE1 potassium channel.
Science,
2002
Jan
18
, 295 (496-9).
1767
Isom LL
The role of sodium channels in cell adhesion.
Front. Biosci.,
2002
Jan
1
, 7 (12-23).
1768
Mason DE
et al.
Molecular basis of voltage-dependent potassium currents in porcine granulosa cells.
Mol. Pharmacol.,
2002
Jan
, 61 (201-13).
1770
Benhorin J
et al.
Variable expression of long QT syndrome among gene carriers from families with five different HERG mutations.
Ann Noninvasive Electrocardiol,
2002
Jan
, 7 (40-6).
1771
Dumaine R
et al.
Molecular mechanisms underlying the long QT syndrome.
Curr. Opin. Cardiol.,
2002
Jan
, 17 (36-42).
1772
Bloise R
et al.
Romano-Ward and other congenital long QT syndromes.
,
2002
Jan
, 16 (19-23).
1773
Moss AJ
et al.
Increased risk of arrhythmic events in long-QT syndrome with mutations in the pore region of the human ether-a-go-go-related gene potassium channel.
Circulation,
2002
Feb
19
, 105 (794-9).
1774
Turnheim K
et al.
Basolateral potassium channels of rabbit colon epithelium: role in sodium absorption and chloride secretion.
Biochim. Biophys. Acta,
2002
Feb
18
, 1560 (51-66).
1775
Ficker E
et al.
The binding site for channel blockers that rescue misprocessed human long QT syndrome type 2 ether-a-gogo-related gene (HERG) mutations.
J. Biol. Chem.,
2002
Feb
15
, 277 (4989-98).
1776
Conrath CE
et al.
Gender differences in the long QT syndrome: effects of beta-adrenoceptor blockade.
Cardiovasc. Res.,
2002
Feb
15
, 53 (770-6).
1777
Cowley EA
et al.
Characterization of basolateral K+ channels underlying anion secretion in the human airway cell line Calu-3.
J. Physiol. (Lond.),
2002
Feb
1
, 538 (747-57).
1779
Ohya S
et al.
Molecular and functional characterization of ERG, KCNQ, and KCNE subtypes in rat stomach smooth muscle.
Am. J. Physiol. Gastrointest. Liver Physiol.,
2002
Feb
, 282 (G277-87).
1780
Kubota T
et al.
Role of KCNQ1 in the cell swelling-induced enhancement of the slowly activating delayed rectifier K(+) current.
Jpn. J. Physiol.,
2002
Feb
, 52 (31-9).
1781
Roti EC
et al.
Interaction with GM130 during HERG ion channel trafficking. Disruption by type 2 congenital long QT syndrome mutations. Human Ether-à-go-go-Related Gene.
J. Biol. Chem.,
2002
Dec
6
, 277 (47779-85).
1782
Peretz A
et al.
Modulation of homomeric and heteromeric KCNQ1 channels by external acidification.
J. Physiol. (Lond.),
2002
Dec
15
, 545 (751-66).
1783
Liu W
et al.
KCNQ1 and KCNH2 mutations associated with long QT syndrome in a Chinese population.
Hum. Mutat.,
2002
Dec
, 20 (475-6).
1784
Wichter T
et al.
Molecular mechanisms of inherited ventricular arrhythmias.
,
2002
Dec
, 27 (712-39).
1785
Roche JP
et al.
Antibodies and a cysteine-modifying reagent show correspondence of M current in neurons to KCNQ2 and KCNQ3 K+ channels.
Br. J. Pharmacol.,
2002
Dec
, 137 (1173-86).
1786
Bosch RF
et al.
beta3-Adrenergic regulation of an ion channel in the heart-inhibition of the slow delayed rectifier potassium current I(Ks) in guinea pig ventricular myocytes.
Cardiovasc. Res.,
2002
Dec
, 56 (393-403).
1787
Cowley EA
et al.
Oxidant stress stimulates anion secretion from the human airway epithelial cell line Calu-3: implications for cystic fibrosis lung disease.
J. Physiol. (Lond.),
2002
Aug
15
, 543 (201-9).
1788
Valdivia CR
et al.
A novel SCN5A arrhythmia mutation, M1766L, with expression defect rescued by mexiletine.
Cardiovasc. Res.,
2002
Aug
1
, 55 (279-89).
1789
Xu X
et al.
Increasing I(Ks) corrects abnormal repolarization in rabbit models of acquired LQT2 and ventricular hypertrophy.
Am. J. Physiol. Heart Circ. Physiol.,
2002
Aug
, 283 (H664-70).
1790
Tristani-Firouzi M
et al.
Functional and clinical characterization of KCNJ2 mutations associated with LQT7 (Andersen syndrome).
J. Clin. Invest.,
2002
Aug
, 110 (381-8).
1791
Isbrandt D
et al.
Identification and functional characterization of a novel KCNE2 (MiRP1) mutation that alters HERG channel kinetics.
J. Mol. Med.,
2002
Aug
, 80 (524-32).
1793
Yang P
et al.
Allelic variants in long-QT disease genes in patients with drug-associated torsades de pointes.
Circulation,
2002
Apr
23
, 105 (1943-8).
1794
Mazhari R
et al.
Ectopic expression of KCNE3 accelerates cardiac repolarization and abbreviates the QT interval.
J. Clin. Invest.,
2002
Apr
, 109 (1083-90).
1795
Wang Z
et al.
Compound heterozygous mutations in KvLQT1 cause Jervell and Lange-Nielsen syndrome.
Mol. Genet. Metab.,
2002
Apr
, 75 (308-16).
1796
Moric E
et al.
The KVLQT1 gene is not a common target for mutations in patients with various heart pathologies.
J. Appl. Genet.,
2002
, 43 (245-54).
1797
Wangemann P
Adrenergic and muscarinic control of cochlear endolymph production.
Adv. Otorhinolaryngol.,
2002
, 59 (42-50).
1798
Towbin JA
Molecular genetic basis of sudden cardiac death.
Cardiovasc. Pathol.,
2001 Nov-Dec
, 10 (283-95).
1799
Kunzelmann K
et al.
Expression and function of colonic epithelial KvLQT1 K+ channels.
Clin. Exp. Pharmacol. Physiol.,
2001 Jan-Feb
, 28 (79-83).
1800
Viswanathan PC
et al.
Gating-dependent mechanisms for flecainide action in SCN5A-linked arrhythmia syndromes.
Circulation,
2001
Sep
4
, 104 (1200-5).
1801
Wedekind H
et al.
De novo mutation in the SCN5A gene associated with early onset of sudden infant death.
Circulation,
2001
Sep
4
, 104 (1158-64).
1802
Schulze-Bahr E
et al.
A novel long-QT 5 gene mutation in the C-terminus (V109I) is associated with a mild phenotype.
J. Mol. Med.,
2001
Sep
, 79 (504-9).
1803
Huang L
et al.
A spectrum of functional effects for disease causing mutations in the Jervell and Lange-Nielsen syndrome.
Cardiovasc. Res.,
2001
Sep
, 51 (670-80).
1804
Wilde AA
et al.
LQT genotype-phenotype relationships: patients and patches.
Cardiovasc. Res.,
2001
Sep
, 51 (627-9).
1805
Nuyens D
et al.
Abrupt rate accelerations or premature beats cause life-threatening arrhythmias in mice with long-QT3 syndrome.
Nat. Med.,
2001
Sep
, 7 (1021-7).
1806
Dedek K
et al.
Colocalization of KCNQ1/KCNE channel subunits in the mouse gastrointestinal tract.
Pflugers Arch.,
2001
Sep
, 442 (896-902).
1807
Seebohm G
et al.
Dependence of I(Ks) biophysical properties on the expression system.
Pflugers Arch.,
2001
Sep
, 442 (891-5).
1808
Tapper AR
et al.
Location and orientation of minK within the I(Ks) potassium channel complex.
J. Biol. Chem.,
2001
Oct
12
, 276 (38249-54).
1809
Franco D
et al.
Divergent expression of delayed rectifier K(+) channel subunits during mouse heart development.
Cardiovasc. Res.,
2001
Oct
, 52 (65-75).
1810
Kang J
et al.
Interactions of the antimalarial drug mefloquine with the human cardiac potassium channels KvLQT1/minK and HERG.
J. Pharmacol. Exp. Ther.,
2001
Oct
, 299 (290-6).
1811
Vallon V
et al.
Role of KCNE1-dependent K+ fluxes in mouse proximal tubule.
J. Am. Soc. Nephrol.,
2001
Oct
, 12 (2003-11).
1812
MacVinish LJ
et al.
Xe991 reveals differences in K(+) channels regulating chloride secretion in murine airway and colonic epithelium.
Mol. Pharmacol.,
2001
Oct
, 60 (753-60).
1813
Perkiömäki JS
et al.
Heart rate variability in patients with congenital long QT syndrome.
Ann Noninvasive Electrocardiol,
2001
Oct
, 6 (298-304).
1814
Kerst G
et al.
Properties and function of KCNQ1 K+ channels isolated from the rectal gland of Squalus acanthias.
Pflugers Arch.,
2001
Oct
, 443 (146-54).
1815
Pond AL
et al.
ERG proteins and functional cardiac I(Kr) channels in rat, mouse, and human heart.
Trends Cardiovasc. Med.,
2001
Oct
, 11 (286-94).
1816
Puchalski RB
et al.
NaCl consumption is attenuated in female KCNE1 null mutant mice.
Physiol. Behav.,
2001
Oct
, 74 (267-76).
1817
Grahammer F
et al.
The small conductance K+ channel, KCNQ1: expression, function, and subunit composition in murine trachea.
J. Biol. Chem.,
2001
Nov
9
, 276 (42268-75).
1818
Furukawa T
et al.
Specific interaction of the potassium channel beta-subunit minK with the sarcomeric protein T-cap suggests a T-tubule-myofibril linking system.
J. Mol. Biol.,
2001
Nov
2
, 313 (775-84).
1819
Larsen LA
et al.
Automated mutation screening using dideoxy fingerprinting and capillary array electrophoresis.
Hum. Mutat.,
2001
Nov
, 18 (451-7).
1820
Ko YL
et al.
Linkage and mutation analysis in two Taiwanese families with long QT syndrome.
J. Formos. Med. Assoc.,
2001
Nov
, 100 (767-71).
1821
Kubota T
et al.
Evidence for a single nucleotide polymorphism in the KCNQ1 potassium channel that underlies susceptibility to life-threatening arrhythmias.
J. Cardiovasc. Electrophysiol.,
2001
Nov
, 12 (1223-9).
1822
Cui J
et al.
Analysis of the cyclic nucleotide binding domain of the HERG potassium channel and interactions with KCNE2.
J. Biol. Chem.,
2001
May
18
, 276 (17244-51).
1823
Pusch M
et al.
Two open states and rate-limiting gating steps revealed by intracellular Na+ block of human KCNQ1 and KCNQ1/KCNE1 K+ channels.
J. Physiol. (Lond.),
2001
May
15
, 533 (135-43).
1824
Li RA
et al.
Functional consequences of the arrhythmogenic G306R KvLQT1 K+ channel mutant probed by viral gene transfer in cardiomyocytes.
J. Physiol. (Lond.),
2001
May
15
, 533 (127-33).
1825
Zhang S
et al.
Cocaine blocks HERG, but not KvLQT1+minK, potassium channels.
Mol. Pharmacol.,
2001
May
, 59 (1069-76).
1826
Grahammer F
et al.
The cardiac K+ channel KCNQ1 is essential for gastric acid secretion.
Gastroenterology,
2001
May
, 120 (1363-71).
1827
Clancy CE
et al.
Cellular consequences of HERG mutations in the long QT syndrome: precursors to sudden cardiac death.
Cardiovasc. Res.,
2001
May
, 50 (301-13).
1828
Kurokawa J
et al.
Molecular basis of the delayed rectifier current I(ks)in heart.
J. Mol. Cell. Cardiol.,
2001
May
, 33 (873-82).
1829
Demolombe S
et al.
Transgenic mice overexpressing human KvLQT1 dominant-negative isoform. Part I: Phenotypic characterisation.
Cardiovasc. Res.,
2001
May
, 50 (314-27).
1830
Lande G
et al.
Transgenic mice overexpressing human KvLQT1 dominant-negative isoform. Part II: Pharmacological profile.
Cardiovasc. Res.,
2001
May
, 50 (328-34).
1832
Potet F
et al.
AKAP proteins anchor cAMP-dependent protein kinase to KvLQT1/IsK channel complex.
Am. J. Physiol. Heart Circ. Physiol.,
2001
May
, 280 (H2038-45).
1833
Chevalier P
et al.
Non-invasive testing of acquired long QT syndrome: evidence for multiple arrhythmogenic substrates.
Cardiovasc. Res.,
2001
May
, 50 (386-98).
1834
Lupoglazoff JM
et al.
[T wave abnormalities on Holter monitoring of congenital long QT syndrome: phenotypic marker of a mutation of LQT2 (HERG)]
Arch Mal Coeur Vaiss,
2001
May
, 94 (470-8).
1835
Nemec J
et al.
Congenital long QT syndromes and Brugada syndrome: the arrhythmogenic ion channel disorders.
,
2001
May
, 2 (773-97).
1836
Kamiya K
et al.
Short- and long-term effects of amiodarone on the two components of cardiac delayed rectifier K(+) current.
Circulation,
2001
Mar
6
, 103 (1317-24).
1837
Korolkova YV
et al.
An ERG channel inhibitor from the scorpion Buthus eupeus.
J. Biol. Chem.,
2001
Mar
30
, 276 (9868-76).
1838
Xiao GQ
et al.
Direct inhibition of expressed cardiac l- and t-type calcium channels by igg from mothers whose children have congenital heart block.
Circulation,
2001
Mar
20
, 103 (1599-604).
1839
Melman YF
et al.
Structural determinants of KvLQT1 control by the KCNE family of proteins.
J. Biol. Chem.,
2001
Mar
2
, 276 (6439-44).
1840
Bliek J
et al.
Increased tumour risk for BWS patients correlates with aberrant H19 and not KCNQ1OT1 methylation: occurrence of KCNQ1OT1 hypomethylation in familial cases of BWS.
Hum. Mol. Genet.,
2001
Mar
1
, 10 (467-76).
1841
Grant AO
Molecular biology of sodium channels and their role in cardiac arrhythmias.
Am. J. Med.,
2001
Mar
, 110 (296-305).
1842
Nicolas M
et al.
KCNQ1/KCNE1 potassium channels in mammalian vestibular dark cells.
Hear. Res.,
2001
Mar
, 153 (132-45).
1843
Han W
et al.
Slow delayed rectifier current and repolarization in canine cardiac Purkinje cells.
Am. J. Physiol. Heart Circ. Physiol.,
2001
Mar
, 280 (H1075-80).
1844
Cerrone M
et al.
[Long QT syndrome and Brugada syndrome: 2 aspects of the same disease?]
,
2001
Mar
, 2 (253-7).
1845
Gaston V
et al.
Analysis of the methylation status of the KCNQ1OT and H19 genes in leukocyte DNA for the diagnosis and prognosis of Beckwith-Wiedemann syndrome.
Eur. J. Hum. Genet.,
2001
Jun
, 9 (409-18).
1846
Bachmann A
et al.
Chromanol 293B, a blocker of the slow delayed rectifier K+ current (IKs), inhibits the CFTR Cl- current.
Naunyn Schmiedebergs Arch. Pharmacol.,
2001
Jun
, 363 (590-6).
1847
Ohyama H
et al.
Inhibition of cardiac delayed rectifier K+ currents by an antisense oligodeoxynucleotide against IsK (minK) and over-expression of IsK mutant D77N in neonatal mouse hearts.
Pflugers Arch.,
2001
Jun
, 442 (329-35).
1848
Lupoglazoff JM
et al.
Homozygous SCN5A mutation in long-QT syndrome with functional two-to-one atrioventricular block.
Circ. Res.,
2001
Jul
20
, 89 (E16-21).
1849
Arrighi I
et al.
Altered potassium balance and aldosterone secretion in a mouse model of human congenital long QT syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
2001
Jul
17
, 98 (8792-7).
1850
Boucherot A
et al.
Regulation and properties of KCNQ1 (K(V)LQT1) and impact of the cystic fibrosis transmembrane conductance regulator.
J. Membr. Biol.,
2001
Jul
1
, 182 (39-47).
1851
Lawson K
et al.
Peripheral channelopathies as targets for potassium channel openers.
,
2001
Jul
, 10 (1345-59).
1852
Miller MD
et al.
Diagnostic accuracy of screening electrocardiograms in long QT syndrome I.
Pediatrics,
2001
Jul
, 108 (8-12).
1853
Schwartz PJ
et al.
Genotype-phenotype correlation in the long-QT syndrome: gene-specific triggers for life-threatening arrhythmias.
Circulation,
2001
Jan
2
, 103 (89-95).
1854
Kunzelmann K
et al.
Cloning and function of the rat colonic epithelial K+ channel KVLQT1.
J. Membr. Biol.,
2001
Jan
15
, 179 (155-64).
1855
Bajanowski T
et al.
Prolonged QT interval and sudden infant death--report of two cases.
Forensic Sci. Int.,
2001
Jan
1
, 115 (147-53).
1856
Kurokawa J
et al.
TEA(+)-sensitive KCNQ1 constructs reveal pore-independent access to KCNE1 in assembled I(Ks) channels.
J. Gen. Physiol.,
2001
Jan
, 117 (43-52).
1857
Kang J
et al.
Interactions of a series of fluoroquinolone antibacterial drugs with the human cardiac K+ channel HERG.
Mol. Pharmacol.,
2001
Jan
, 59 (122-6).
1858
Paul AA
et al.
Inhibition of HERG potassium channel current by the class 1a antiarrhythmic agent disopyramide.
Biochem. Biophys. Res. Commun.,
2001
Feb
9
, 280 (1243-50).
1859
Lupoglazoff JM
et al.
Notched T waves on Holter recordings enhance detection of patients with LQt2 (HERG) mutations.
Circulation,
2001
Feb
27
, 103 (1095-101).
1860
Casimiro MC
et al.
Targeted disruption of the Kcnq1 gene produces a mouse model of Jervell and Lange-Nielsen Syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
2001
Feb
27
, 98 (2526-31).
1861
Demolombe S
et al.
Differential expression of KvLQT1 and its regulator IsK in mouse epithelia.
Am. J. Physiol., Cell Physiol.,
2001
Feb
, 280 (C359-72).
1862
Piippo K
et al.
A founder mutation of the potassium channel KCNQ1 in long QT syndrome: implications for estimation of disease prevalence and molecular diagnostics.
J. Am. Coll. Cardiol.,
2001
Feb
, 37 (562-8).
1863
Yamashita F
et al.
Characterization and subcellular localization of KCNQ1 with a heterozygous mutation in the C terminus.
J. Mol. Cell. Cardiol.,
2001
Feb
, 33 (197-207).
1864
Weksberg R
et al.
Tumor development in the Beckwith-Wiedemann syndrome is associated with a variety of constitutional molecular 11p15 alterations including imprinting defects of KCNQ1OT1.
Hum. Mol. Genet.,
2001
Dec
15
, 10 (2989-3000).
1865
Lee JH
et al.
Effect of vasopressin on marginal cells of neonatal rat cochlea in vitro.
Acta Otolaryngol.,
2001
Dec
, 121 (902-7).
1866
Seebohm G
et al.
A kinetic study on the stereospecific inhibition of KCNQ1 and I(Ks) by the chromanol 293B.
Br. J. Pharmacol.,
2001
Dec
, 134 (1647-54).
1867
Huang FD
et al.
Long-QT syndrome-associated missense mutations in the pore helix of the HERG potassium channel.
Circulation,
2001
Aug
28
, 104 (1071-5).
1868
Rivolta I
et al.
Inherited Brugada and long QT-3 syndrome mutations of a single residue of the cardiac sodium channel confer distinct channel and clinical phenotypes.
J. Biol. Chem.,
2001
Aug
17
, 276 (30623-30).
1869
Tatulian L
et al.
Activation of expressed KCNQ potassium currents and native neuronal M-type potassium currents by the anti-convulsant drug retigabine.
J. Neurosci.,
2001
Aug
1
, 21 (5535-45).
1870
Larsen LA
et al.
Screening for mutations and polymorphisms in the genes KCNH2 and KCNE2 encoding the cardiac HERG/MiRP1 ion channel: implications for acquired and congenital long Q-T syndrome.
Clin. Chem.,
2001
Aug
, 47 (1390-5).
1871
Seebohm G
et al.
Identification of specific pore residues mediating KCNQ1 inactivation. A novel mechanism for long QT syndrome.
J. Biol. Chem.,
2001
Apr
27
, 276 (13600-5).
1872
Hoppe UC
et al.
Distinct gene-specific mechanisms of arrhythmia revealed by cardiac gene transfer of two long QT disease genes, HERG and KCNE1.
Proc. Natl. Acad. Sci. U.S.A.,
2001
Apr
24
, 98 (5335-40).
1873
Abriel H
et al.
Novel arrhythmogenic mechanism revealed by a long-QT syndrome mutation in the cardiac Na(+) channel.
Circ. Res.,
2001
Apr
13
, 88 (740-5).
1874
Chabannes D
et al.
Mice disrupted for the KvLQT1 potassium channel regulator IsK gene accumulate mature T cells.
Cell. Immunol.,
2001
Apr
10
, 209 (1-9).
1875
Windle JR
et al.
Normalization of ventricular repolarization with flecainide in long QT syndrome patients with SCN5A:DeltaKPQ mutation.
Ann Noninvasive Electrocardiol,
2001
Apr
, 6 (153-8).
1876
Caballero R
et al.
Direct effects of candesartan and eprosartan on human cloned potassium channels involved in cardiac repolarization.
Mol. Pharmacol.,
2001
Apr
, 59 (825-36).
1877
Robbins J
KCNQ potassium channels: physiology, pathophysiology, and pharmacology.
Pharmacol. Ther.,
2001
Apr
, 90 (1-19).
1878
Moennig G
et al.
Clinical value of electrocardiographic parameters in genotyped individuals with familial long QT syndrome.
,
2001
Apr
, 24 (406-15).
1879
Towbin JA
et al.
Genotype and severity of long QT syndrome.
Drug Metab. Dispos.,
2001
Apr
, 29 (574-9).
1880
Søgaard R
et al.
KCNQ4 channels expressed in mammalian cells: functional characteristics and pharmacology.
Am. J. Physiol., Cell Physiol.,
2001
Apr
, 280 (C859-66).
1881
Kaufman ES
et al.
Electrocardiographic prediction of abnormal genotype in congenital long QT syndrome: experience in 101 related family members.
J. Cardiovasc. Electrophysiol.,
2001
Apr
, 12 (455-61).
1882
Allan WC
et al.
Long QT syndrome in children: the value of rate corrected QT interval and DNA analysis as screening tests in the general population.
,
2001
, 8 (173-7).
1883
Antzelevitch C
Molecular biology and cellular mechanisms of Brugada and long QT syndromes in infants and young children.
,
2001
, 34 Suppl (177-81).
1884
Tanaka K
et al.
Loss of imprinting of long QT intronic transcript 1 in colorectal cancer.
Oncology,
2001
, 60 (268-73).
1885
Dell G
et al.
In vitro methylation of specific regions in recombinant DNA constructs by excision and religation.
Methods Mol. Biol.,
2001
, 181 (251-8).
1886
Li H
et al.
Current concepts in long QT syndrome.
Pediatr Cardiol,
2000 Nov-Dec
, 21 (542-50).
1887
Drici MD
et al.
Cardiac K+ channels and drug-acquired long QT syndrome.
Therapie,
2000 Jan-Feb
, 55 (185-93).
1888
Chouabe C
et al.
Effects of calcium channel blockers on cloned cardiac K+ channels IKr and IKs.
Therapie,
2000 Jan-Feb
, 55 (195-202).
1889
Georgijević Milić L
[Molecular genetics in the hereditary form of long QT syndrome]
Med. Pregl.,
2000 Jan-Feb
, 53 (51-4).
1890
Makita N
et al.
Cardiac Na(+) channel dysfunction in Brugada syndrome is aggravated by beta(1)-subunit.
Circulation,
2000 Jan 4-11
, 101 (54-60).
1891
Nakajima T
et al.
Characterization of S818L mutation in HERG C-terminus in LQT2. Modification of activation-deactivation gating properties.
FEBS Lett.,
2000
Sep
15
, 481 (197-203).
1892
Sesti F
et al.
A common polymorphism associated with antibiotic-induced cardiac arrhythmia.
Proc. Natl. Acad. Sci. U.S.A.,
2000
Sep
12
, 97 (10613-8).
1893
Kathöfer S
et al.
Functional coupling of human beta 3-adrenoreceptors to the KvLQT1/MinK potassium channel.
J. Biol. Chem.,
2000
Sep
1
, 275 (26743-7).
1894
Freeman LC
et al.
Glycosylation influences gating and pH sensitivity of I(sK).
J. Membr. Biol.,
2000
Sep
1
, 177 (65-79).
1895
Tapper AR
et al.
MinK subdomains that mediate modulation of and association with KvLQT1.
J. Gen. Physiol.,
2000
Sep
, 116 (379-90).
1896
Kubota T
et al.
Hypokalemia-induced long QT syndrome with an underlying novel missense mutation in S4-S5 linker of KCNQ1.
J. Cardiovasc. Electrophysiol.,
2000
Sep
, 11 (1048-54).
1897
Sesti F
et al.
MinK endows the I(Ks) potassium channel pore with sensitivity to internal tetraethylammonium.
Biophys. J.,
2000
Sep
, 79 (1369-78).
1898
Yang IC
et al.
Stereoselective interactions of the enantiomers of chromanol 293B with human voltage-gated potassium channels.
J. Pharmacol. Exp. Ther.,
2000
Sep
, 294 (955-62).
1899
Mall M
et al.
Role of K(V)LQT1 in cyclic adenosine monophosphate-mediated Cl(-) secretion in human airway epithelia.
Am. J. Respir. Cell Mol. Biol.,
2000
Sep
, 23 (283-9).
1900
Mitcheson JS
et al.
A structural basis for drug-induced long QT syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
2000
Oct
24
, 97 (12329-33).
1901
Jiang M
et al.
Delayed rectifier K currents have reduced amplitudes and altered kinetics in myocytes from infarcted canine ventricle.
Cardiovasc. Res.,
2000
Oct
, 48 (34-43).
1902
Witchel HJ
et al.
Familial and acquired long qt syndrome and the cardiac rapid delayed rectifier potassium current.
Clin. Exp. Pharmacol. Physiol.,
2000
Oct
, 27 (753-66).
1903
Ficker E
et al.
Novel characteristics of a misprocessed mutant HERG channel linked to hereditary long QT syndrome.
Am. J. Physiol. Heart Circ. Physiol.,
2000
Oct
, 279 (H1748-56).
1904
Lock H
et al.
Contribution of the IsK (MinK) potassium channel subunit to regulatory volume decrease in murine tracheal epithelial cells.
J. Biol. Chem.,
2000
Nov
10
, 275 (34849-52).
1905
Engemann S
et al.
Sequence and functional comparison in the Beckwith-Wiedemann region: implications for a novel imprinting centre and extended imprinting.
Hum. Mol. Genet.,
2000
Nov
1
, 9 (2691-706).
1906
Tyson J
et al.
Mutational spectrum in the cardioauditory syndrome of Jervell and Lange-Nielsen.
Hum. Genet.,
2000
Nov
, 107 (499-503).
1907
Xin Z
et al.
A novel imprinted gene, KCNQ1DN, within the WT2 critical region of human chromosome 11p15.5 and its reduced expression in Wilms' tumors.
J. Biochem.,
2000
Nov
, 128 (847-53).
1908
Kuryshev YA
et al.
Interactions of the 5-hydroxytryptamine 3 antagonist class of antiemetic drugs with human cardiac ion channels.
J. Pharmacol. Exp. Ther.,
2000
Nov
, 295 (614-20).
1909
Veldkamp MW
et al.
Two distinct congenital arrhythmias evoked by a multidysfunctional Na(+) channel.
Circ. Res.,
2000
May
12
, 86 (E91-7).
1910
Bowles NE
et al.
The "final common pathway" hypothesis and inherited cardiovascular disease. The role of cytoskeletal proteins in dilated cardiomyopathy.
,
2000
May
, 25 (168-75).
1911
Paulussen A
et al.
Analysis of the human KCNH2(HERG) gene: identification and characterization of a novel mutation Y667X associated with long QT syndrome and a non-pathological 9 bp insertion.
Hum. Mutat.,
2000
May
, 15 (483).
1913
Tamargo J
Drug-induced torsade de pointes: from molecular biology to bedside.
Jpn. J. Pharmacol.,
2000
May
, 83 (1-19).
1914
Hayashi K
et al.
Identical twins with long QT syndrome associated with a missense mutation in the S4 region of the HERG.
,
2000
May
, 41 (399-404).
1915
Stramba-Badiale M
et al.
Gene-specific differences in the circadian variation of ventricular repolarization in the long QT syndrome: a key to sudden death during sleep?
Ital Heart J,
2000
May
, 1 (323-8).
1916
Kuryshev YA
et al.
KChAP as a chaperone for specific K(+) channels.
Am. J. Physiol., Cell Physiol.,
2000
May
, 278 (C931-41).
1917
Kang J
et al.
High affinity blockade of the HERG cardiac K(+) channel by the neuroleptic pimozide.
Eur. J. Pharmacol.,
2000
Mar
31
, 392 (137-40).
1918
Robertson GA
LQT2 : amplitude reduction and loss of selectivity in the tail that wags the HERG channel.
Circ. Res.,
2000
Mar
17
, 86 (492-3).
1919
Lees-Miller JP
et al.
Novel gain-of-function mechanism in K(+) channel-related long-QT syndrome: altered gating and selectivity in the HERG1 N629D mutant.
Circ. Res.,
2000
Mar
17
, 86 (507-13).
1920
Wulfsen I
et al.
Expression of mRNA for voltage-dependent and inward-rectifying K channels in GH3/B6 cells and rat pituitary.
J. Neuroendocrinol.,
2000
Mar
, 12 (263-72).
1921
Chouabe C
et al.
Novel mutations in KvLQT1 that affect Iks activation through interactions with Isk.
Cardiovasc. Res.,
2000
Mar
, 45 (971-80).
1922
Sperandeo MP
et al.
Relaxation of insulin-like growth factor 2 imprinting and discordant methylation at KvDMR1 in two first cousins affected by Beckwith-Wiedemann and Klippel-Trenaunay-Weber syndromes.
Am. J. Hum. Genet.,
2000
Mar
, 66 (841-7).
1923
Yatsuki H
et al.
Sequence-based structural features between Kvlqt1 and Tapa1 on mouse chromosome 7F4/F5 corresponding to the Beckwith-Wiedemann syndrome region on human 11p15.5: long-stretches of unusually well conserved intronic sequences of kvlqt1 between mouse and huma
DNA Res.,
2000
Jun
30
, 7 (195-206).
1924
Cui J
et al.
Cyclic AMP regulates the HERG K(+) channel by dual pathways.
Curr. Biol.,
2000
Jun
1
, 10 (671-4).
1925
Laitinen P
et al.
Survey of the coding region of the HERG gene in long QT syndrome reveals six novel mutations and an amino acid polymorphism with possible phenotypic effects.
Hum. Mutat.,
2000
Jun
, 15 (580-1).
1926
Péréon Y
et al.
Differential expression of KvLQT1 isoforms across the human ventricular wall.
Am. J. Physiol. Heart Circ. Physiol.,
2000
Jun
, 278 (H1908-15).
1927
Piippo K
et al.
Homozygosity for a HERG potassium channel mutation causes a severe form of long QT syndrome: identification of an apparent founder mutation in the Finns.
J. Am. Coll. Cardiol.,
2000
Jun
, 35 (1919-25).
1928
Bleich M
et al.
The very small-conductance K+ channel KvLQT1 and epithelial function.
Pflugers Arch.,
2000
Jun
, 440 (202-6).
1929
Postma AV
et al.
Genomic organisation and chromosomal localisation of two members of the KCND ion channel family, KCND2 and KCND3.
Hum. Genet.,
2000
Jun
, 106 (614-9).
1930
Wang HS
et al.
Molecular basis for differential sensitivity of KCNQ and I(Ks) channels to the cognitive enhancer XE991.
Mol. Pharmacol.,
2000
Jun
, 57 (1218-23).
1931
Napolitano C
et al.
Evidence for a cardiac ion channel mutation underlying drug-induced QT prolongation and life-threatening arrhythmias.
J. Cardiovasc. Electrophysiol.,
2000
Jun
, 11 (691-6).
1932
Sanguinetti MC
Long QT syndrome: ionic basis and arrhythmia mechanism in long QT syndrome type 1.
J. Cardiovasc. Electrophysiol.,
2000
Jun
, 11 (710-2).
1933
Enklaar T
et al.
Mtr1, a novel biallelically expressed gene in the center of the mouse distal chromosome 7 imprinting cluster, is a member of the Trp gene family.
Genomics,
2000
Jul
15
, 67 (179-87).
1934
Bennett PB
Long QT syndrome: biophysical and pharmacologic mechanisms in LQT3.
J. Cardiovasc. Electrophysiol.,
2000
Jul
, 11 (819-22).
1935
Chiang CE
et al.
The long QT syndromes: genetic basis and clinical implications.
J. Am. Coll. Cardiol.,
2000
Jul
, 36 (1-12).
1936
Huber SM
et al.
Metanephrogenic mesenchyme-to-epithelium transition induces profound expression changes of ion channels.
Am. J. Physiol. Renal Physiol.,
2000
Jul
, 279 (F65-76).
1937
Katayama Y
et al.
Inhibitory effects of vesnarinone on cloned cardiac delayed rectifier K(+) channels expressed in a mammalian cell line.
J. Pharmacol. Exp. Ther.,
2000
Jul
, 294 (339-46).
1938
Krahn AD
et al.
A novel mutation in KVLQT1, L122P, found in a family with autosomal dominant long QT syndrome.
Am. Heart J.,
2000
Jul
, 140 (146-9).
1939
Prawitt D
et al.
Identification and characterization of MTR1, a novel gene with homology to melastatin (MLSN1) and the trp gene family located in the BWS-WT2 critical region on chromosome 11p15.5 and showing allele-specific expression.
Hum. Mol. Genet.,
2000
Jan
22
, 9 (203-16).
1940
Schroeder BC
et al.
A constitutively open potassium channel formed by KCNQ1 and KCNE3.
Nature,
2000
Jan
13
, 403 (196-9).
1941
Pusch M
et al.
Gating and flickery block differentially affected by rubidium in homomeric KCNQ1 and heteromeric KCNQ1/KCNE1 potassium channels.
Biophys. J.,
2000
Jan
, 78 (211-26).
1942
Nagatomo T
et al.
Preferential block of late sodium current in the LQT3 DeltaKPQ mutant by the class I(C) antiarrhythmic flecainide.
Mol. Pharmacol.,
2000
Jan
, 57 (101-7).
1943
Pond AL
et al.
Expression of distinct ERG proteins in rat, mouse, and human heart. Relation to functional I(Kr) channels.
J. Biol. Chem.,
2000
Feb
25
, 275 (5997-6006).
1944
Schmitt N
et al.
A recessive C-terminal Jervell and Lange-Nielsen mutation of the KCNQ1 channel impairs subunit assembly.
EMBO J.,
2000
Feb
1
, 19 (332-40).
1945
Selyanko AA
et al.
Inhibition of KCNQ1-4 potassium channels expressed in mammalian cells via M1 muscarinic acetylcholine receptors.
J. Physiol. (Lond.),
2000
Feb
1
, 522 Pt 3 (349-55).
1946
Hadley JK
et al.
Differential tetraethylammonium sensitivity of KCNQ1-4 potassium channels.
Br. J. Pharmacol.,
2000
Feb
, 129 (413-5).
1947
Baroudi G
et al.
Biophysical phenotypes of SCN5A mutations causing long QT and Brugada syndromes.
FEBS Lett.,
2000
Dec
29
, 487 (224-8).
1948
Tinel N
et al.
KCNE2 confers background current characteristics to the cardiac KCNQ1 potassium channel.
EMBO J.,
2000
Dec
1
, 19 (6326-30).
1949
Ficker E
et al.
Retention in the endoplasmic reticulum as a mechanism of dominant-negative current suppression in human long QT syndrome.
J. Mol. Cell. Cardiol.,
2000
Dec
, 32 (2327-37).
1950
Engel JR
et al.
Epigenotype-phenotype correlations in Beckwith-Wiedemann syndrome.
J. Med. Genet.,
2000
Dec
, 37 (921-6).
1951
Lerche C
et al.
Molecular impact of MinK on the enantiospecific block of I(Ks) by chromanols.
Br. J. Pharmacol.,
2000
Dec
, 131 (1503-6).
1952
Bianchi L
et al.
Mechanisms of I(Ks) suppression in LQT1 mutants.
Am. J. Physiol. Heart Circ. Physiol.,
2000
Dec
, 279 (H3003-11).
1953
Miraglia del Giudice E
et al.
Benign familial neonatal convulsions (BFNC) resulting from mutation of the KCNQ2 voltage sensor.
Eur. J. Hum. Genet.,
2000
Dec
, 8 (994-7).
1954
Novotný T
et al.
[The role of ergometric testing in diagnosis of latent long QT syndrome]
,
2000
Dec
, 46 (843-7).
1955
Unsöld B
et al.
KCNE1 reverses the response of the human K+ channel KCNQ1 to cytosolic pH changes and alters its pharmacology and sensitivity to temperature.
Pflugers Arch.,
2000
Dec
, 441 (368-78).
1956
January CT
et al.
Long QT syndrome: cellular basis and arrhythmia mechanism in LQT2.
J. Cardiovasc. Electrophysiol.,
2000
Dec
, 11 (1413-8).
1957
Lee MP
et al.
Targeted disruption of the Kvlqt1 gene causes deafness and gastric hyperplasia in mice.
J. Clin. Invest.,
2000
Dec
, 106 (1447-55).
1958
Shimizu W
et al.
Effects of a K(+) channel opener to reduce transmural dispersion of repolarization and prevent torsade de pointes in LQT1, LQT2, and LQT3 models of the long-QT syndrome.
Circulation,
2000
Aug
8
, 102 (706-12).
1959
Priori SG
et al.
The elusive link between LQT3 and Brugada syndrome: the role of flecainide challenge.
Circulation,
2000
Aug
29
, 102 (945-7).
1960
Abriel H
et al.
Molecular pharmacology of the sodium channel mutation D1790G linked to the long-QT syndrome.
Circulation,
2000
Aug
22
, 102 (921-5).
1961
Wehrens XH
et al.
Arrhythmogenic mechanism of an LQT-3 mutation of the human heart Na(+) channel alpha-subunit: A computational analysis.
Circulation,
2000
Aug
1
, 102 (584-90).
1963
Mall M
et al.
Effect of genistein on native epithelial tissue from normal individuals and CF patients and on ion channels expressed in Xenopus oocytes.
Br. J. Pharmacol.,
2000
Aug
, 130 (1884-92).
1964
Yamashita T
et al.
Short-term effects of rapid pacing on mRNA level of voltage-dependent K(+) channels in rat atrium: electrical remodeling in paroxysmal atrial tachycardia.
Circulation,
2000
Apr
25
, 101 (2007-14).
1965
Kagan A
et al.
The dominant negative LQT2 mutation A561V reduces wild-type HERG expression.
J. Biol. Chem.,
2000
Apr
14
, 275 (11241-8).
1966
Wei J
et al.
Novel KCNQ1 mutations associated with recessive and dominant congenital long QT syndromes: evidence for variable hearing phenotype associated with R518X.
Hum. Mutat.,
2000
Apr
, 15 (387-8).
1967
Leenhardt A
et al.
[Present concepts of congenital long QT syndrome]
Arch Mal Coeur Vaiss,
2000
Apr
, 93 (17-21).
1968
Burashnikov A
et al.
Block of I(Ks) does not induce early afterdepolarization activity but promotes beta-adrenergic agonist-induced delayed afterdepolarization activity.
J. Cardiovasc. Electrophysiol.,
2000
Apr
, 11 (458-65).
1969
Kambouris NG
et al.
A revised view of cardiac sodium channel "blockade" in the long-QT syndrome.
J. Clin. Invest.,
2000
Apr
, 105 (1133-40).
1970
Dai DZ
Vulnerable substrate and multiple ion channel disorder in a diseased heart will be new targets for antiarrhythmic therapy.
Acta Pharmacol. Sin.,
2000
Apr
, 21 (289-95).
1971
Deschênes I
et al.
Electrophysiological characterization of SCN5A mutations causing long QT (E1784K) and Brugada (R1512W and R1432G) syndromes.
Cardiovasc. Res.,
2000
Apr
, 46 (55-65).
1972
Akar FG
et al.
Cellular basis for dispersion of repolarization underlying reentrant arrhythmias.
,
2000
, 33 Suppl (23-31).
1973
Schulze-Bahr E
et al.
Molecular genetics of arrhythmias--a new paradigm.
,
2000
, 89 Suppl 4 (IV12-22).
1975
Weirich J
et al.
[Current classification of anti-arrhythmia agents]
,
2000
, 89 Suppl 3 (62-7).
1976
Vincent GM
et al.
The inherited long QT syndrome: from ion channel to bedside.
,
1999 Jan-Feb
, 7 (44-55).
1977
Bezzina C
et al.
A single Na(+) channel mutation causing both long-QT and Brugada syndromes.
Circ. Res.,
1999 Dec 3-17
, 85 (1206-13).
1978
Murray A
et al.
Splicing mutations in KCNQ1: a mutation hot spot at codon 344 that produces in frame transcripts.
Circulation,
1999
Sep
7
, 100 (1077-84).
1979
Tranebjaerg L
et al.
Jervell and Lange-Nielsen syndrome: a Norwegian perspective.
Am. J. Med. Genet.,
1999
Sep
24
, 89 (137-46).
1980
Hoorntje T
et al.
Homozygous premature truncation of the HERG protein : the human HERG knockout.
Circulation,
1999
Sep
21
, 100 (1264-7).
1981
Piccini M
et al.
KCNE1-like gene is deleted in AMME contiguous gene syndrome: identification and characterization of the human and mouse homologs.
Genomics,
1999
Sep
15
, 60 (251-7).
1982
Larsen LA
et al.
Recessive Romano-Ward syndrome associated with compound heterozygosity for two mutations in the KVLQT1 gene.
Eur. J. Hum. Genet.,
1999
Sep
, 7 (724-8).
1983
Swan H
et al.
Sinus node function and ventricular repolarization during exercise stress test in long QT syndrome patients with KvLQT1 and HERG potassium channel defects.
J. Am. Coll. Cardiol.,
1999
Sep
, 34 (823-9).
1984
Yoshida H
et al.
Characterization of a novel missense mutation in the pore of HERG in a patient with long QT syndrome.
J. Cardiovasc. Electrophysiol.,
1999
Sep
, 10 (1262-70).
1985
Warth R
et al.
Molecular and functional characterization of the small Ca(2+)-regulated K+ channel (rSK4) of colonic crypts.
Pflugers Arch.,
1999
Sep
, 438 (437-44).
1986
Lee-Chen GJ
et al.
Romano-Ward long QT syndrome: identification of a HERG mutation in a Taiwanese kindred.
J. Formos. Med. Assoc.,
1999
Sep
, 98 (649-52).
1987
Zhou Z
et al.
Correction of defective protein trafficking of a mutant HERG potassium channel in human long QT syndrome. Pharmacological and temperature effects.
J. Biol. Chem.,
1999
Oct
29
, 274 (31123-6).
1988
Wattanasirichaigoon D
et al.
Sodium channel abnormalities are infrequent in patients with long QT syndrome: identification of two novel SCN5A mutations.
Am. J. Med. Genet.,
1999
Oct
29
, 86 (470-6).
1989
Köttgen M
et al.
Carbachol activates a K+ channel of very small conductance in the basolateral membrane of rat pancreatic acinar cells.
Pflugers Arch.,
1999
Oct
, 438 (597-603).
1990
Lehmann-Horn F
et al.
Voltage-gated ion channels and hereditary disease.
Physiol. Rev.,
1999
Oct
, 79 (1317-72).
1992
Sepp R
et al.
[Molecular genetics of the long QT syndrome: clinical aspects]
,
1999
Nov
21
, 140 (2633-8).
1993
Couderc JP
et al.
Beat-to-Beat repolarization variability in LQTS patients with the SCN5A sodium channel gene mutation.
,
1999
Nov
, 22 (1581-92).
1994
Nakajima T
et al.
Voltage-shift of the current activation in HERG S4 mutation (R534C) in LQT2.
Cardiovasc. Res.,
1999
Nov
, 44 (283-93).
1995
Furutani M
et al.
Novel mechanism associated with an inherited cardiac arrhythmia: defective protein trafficking by the mutant HERG (G601S) potassium channel.
Circulation,
1999
May
4
, 99 (2290-4).
1997
Viswanathan PC
et al.
Pause induced early afterdepolarizations in the long QT syndrome: a simulation study.
Cardiovasc. Res.,
1999
May
, 42 (530-42).
1998
Shimizu W
et al.
Cellular and ionic basis for T-wave alternans under long-QT conditions.
Circulation,
1999
Mar
23
, 99 (1499-507).
1999
Berthet M
et al.
C-terminal HERG mutations: the role of hypokalemia and a KCNQ1-associated mutation in cardiac event occurrence.
Circulation,
1999
Mar
23
, 99 (1464-70).
2000
Chen Q
et al.
Homozygous deletion in KVLQT1 associated with Jervell and Lange-Nielsen syndrome.
Circulation,
1999
Mar
16
, 99 (1344-7).
2001
Wei J
et al.
Congenital long-QT syndrome caused by a novel mutation in a conserved acidic domain of the cardiac Na+ channel.
Circulation,
1999
Jun
22
, 99 (3165-71).
2002
Bou-Abboud E
et al.
Molecular correlates of the calcium-independent, depolarization-activated K+ currents in rat atrial myocytes.
J. Physiol. (Lond.),
1999
Jun
1
, 517 ( Pt 2) (407-20).
2003
Wang Z
et al.
Functional effects of mutations in KvLQT1 that cause long QT syndrome.
J. Cardiovasc. Electrophysiol.,
1999
Jun
, 10 (817-26).
2004
Smilinich NJ
et al.
A maternally methylated CpG island in KvLQT1 is associated with an antisense paternal transcript and loss of imprinting in Beckwith-Wiedemann syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
1999
Jul
6
, 96 (8064-9).
2005
Franqueza L
et al.
Long QT syndrome-associated mutations in the S4-S5 linker of KvLQT1 potassium channels modify gating and interaction with minK subunits.
J. Biol. Chem.,
1999
Jul
23
, 274 (21063-70).
2006
Mitsuya K
et al.
LIT1, an imprinted antisense RNA in the human KvLQT1 locus identified by screening for differentially expressed transcripts using monochromosomal hybrids.
Hum. Mol. Genet.,
1999
Jul
, 8 (1209-17).
2007
Vizgirda VM
The genetic basis for cardiac dysrhythmias and the long QT syndrome.
,
1999
Jul
, 13 (34-45).
2008
Diochot S
et al.
Effects of phrixotoxins on the Kv4 family of potassium channels and implications for the role of Ito1 in cardiac electrogenesis.
Br. J. Pharmacol.,
1999
Jan
, 126 (251-63).
2009
Waldegger S
et al.
Molecular and functional characterization of s-KCNQ1 potassium channel from rectal gland of Squalus acanthias.
Pflugers Arch.,
1999
Jan
, 437 (298-304).
2010
El-Sherif N
et al.
The long QT syndrome and torsade de pointes.
Pacing Clin Electrophysiol,
1999
Jan
, 22 (91-110).
2011
Kubisch C
et al.
KCNQ4, a novel potassium channel expressed in sensory outer hair cells, is mutated in dominant deafness.
Cell,
1999
Feb
5
, 96 (437-46).
2012
Neyroud N
et al.
Genomic organization of the KCNQ1 K+ channel gene and identification of C-terminal mutations in the long-QT syndrome.
Circ. Res.,
1999
Feb
19
, 84 (290-7).
2013
Petrecca K
et al.
N-linked glycosylation sites determine HERG channel surface membrane expression.
J. Physiol. (Lond.),
1999
Feb
15
, 515 ( Pt 1) (41-8).
2014
Larsen LA
et al.
A single strand conformation polymorphism/heteroduplex (SSCP/HD) method for detection of mutations in 15 exons of the KVLQT1 gene, associated with long QT syndrome.
Clin. Chim. Acta,
1999
Feb
, 280 (113-25).
2015
Tan HL
et al.
Long-term (subacute) potassium treatment in congenital HERG-related long QT syndrome (LQTS2).
J. Cardiovasc. Electrophysiol.,
1999
Feb
, 10 (229-33).
2016
Po SS
et al.
Modulation of HERG potassium channels by extracellular magnesium and quinidine.
J. Cardiovasc. Pharmacol.,
1999
Feb
, 33 (181-5).
2017
Wilde AA
et al.
Auditory stimuli as a trigger for arrhythmic events differentiate HERG-related (LQTS2) patients from KVLQT1-related patients (LQTS1).
J. Am. Coll. Cardiol.,
1999
Feb
, 33 (327-32).
2018
Eaholtz G
et al.
Block of brain sodium channels by peptide mimetics of the isoleucine, phenylalanine, and methionine (IFM) motif from the inactivation gate.
J. Gen. Physiol.,
1999
Feb
, 113 (279-94).
2019
Shimizu W
et al.
Sodium pentobarbital reduces transmural dispersion of repolarization and prevents torsades de Pointes in models of acquired and congenital long QT syndrome.
J. Cardiovasc. Electrophysiol.,
1999
Feb
, 10 (154-64).
2020
Roden DM
et al.
Inherited long QT syndromes: a paradigm for understanding arrhythmogenesis.
J. Cardiovasc. Electrophysiol.,
1999
Dec
, 10 (1664-83).
2021
Abitbol I
et al.
Stilbenes and fenamates rescue the loss of I(KS) channel function induced by an LQT5 mutation and other IsK mutants.
EMBO J.,
1999
Aug
2
, 18 (4137-48).
2022
Bianchi L
et al.
Cellular dysfunction of LQT5-minK mutants: abnormalities of IKs, IKr and trafficking in long QT syndrome.
Hum. Mol. Genet.,
1999
Aug
, 8 (1499-507).
2023
Sanguinetti MC
Dysfunction of delayed rectifier potassium channels in an inherited cardiac arrhythmia.
Ann. N. Y. Acad. Sci.,
1999
Apr
30
, 868 (406-13).
2024
Lee MP
et al.
Loss of imprinting of a paternally expressed transcript, with antisense orientation to KVLQT1, occurs frequently in Beckwith-Wiedemann syndrome and is independent of insulin-like growth factor II imprinting.
Proc. Natl. Acad. Sci. U.S.A.,
1999
Apr
27
, 96 (5203-8).
2025
Matsuoka R
et al.
A mitochondrial DNA mutation cosegregates with the pathophysiological U wave.
Biochem. Biophys. Res. Commun.,
1999
Apr
2
, 257 (228-33).
2026
Schulze-Bahr E
et al.
The LQT syndromes--current status of molecular mechanisms.
,
1999
Apr
, 88 (245-54).
2027
Mohammad-Panah R
et al.
Mutations in a dominant-negative isoform correlate with phenotype in inherited cardiac arrhythmias.
Am. J. Hum. Genet.,
1999
Apr
, 64 (1015-23).
2028
Jongbloed RJ
et al.
Novel KCNQ1 and HERG missense mutations in Dutch long-QT families.
Hum. Mutat.,
1999
, 13 (301-10).
2029
Suessbrich H
et al.
The IKs channel: coassembly of IsK (minK) and KvLQT1 proteins.
Rev. Physiol. Biochem. Pharmacol.,
1999
, 137 (191-226).
2030
Kunzelmann K
The cystic fibrosis transmembrane conductance regulator and its function in epithelial transport.
Rev. Physiol. Biochem. Pharmacol.,
1999
, 137 (1-70).
2031
Shimizu W
et al.
Cellular basis for long QT, transmural dispersion of repolarization, and torsade de pointes in the long QT syndrome.
,
1999
, 32 Suppl (177-84).
2032
Antzelevitch C
et al.
Transmural dispersion of repolarization and arrhythmogenicity: the Brugada syndrome versus the long QT syndrome.
,
1999
, 32 Suppl (158-65).
2033
Mitcheson JS
et al.
Biophysical properties and molecular basis of cardiac rapid and slow delayed rectifier potassium channels.
Cell. Physiol. Biochem.,
1999
, 9 (201-16).
2034
Lai LP
et al.
Changes in the mRNA levels of delayed rectifier potassium channels in human atrial fibrillation.
Cardiology,
1999
, 92 (248-55).
2035
Neyroud N
et al.
Heterozygous mutation in the pore of potassium channel gene KvLQT1 causes an apparently normal phenotype in long QT syndrome.
Eur. J. Hum. Genet.,
1998 Mar-Apr
, 6 (129-33).
2036
Viskin S
et al.
Polymorphic ventricular tachyarrhythmias in the absence of organic heart disease: classification, differential diagnosis, and implications for therapy.
,
1998 Jul-Aug
, 41 (17-34).
2037
Babij P
et al.
Inhibition of cardiac delayed rectifier K+ current by overexpression of the long-QT syndrome HERG G628S mutation in transgenic mice.
Circ. Res.,
1998
Sep
21
, 83 (668-78).
2038
Shen Z
et al.
Divalent cations inhibit IsK/KvLQT1 channels in excised membrane patches of strial marginal cells.
Hear. Res.,
1998
Sep
, 123 (157-67).
2039
Wang Q
et al.
The molecular basis of long QT syndrome and prospects for therapy.
,
1998
Sep
, 4 (382-8).
2040
Itoh T
et al.
Genomic organization and mutational analysis of KVLQT1, a gene responsible for familial long QT syndrome.
Hum. Genet.,
1998
Sep
, 103 (290-4).
2041
Charpentier F
et al.
Adult KCNE1-knockout mice exhibit a mild cardiac cellular phenotype.
Biochem. Biophys. Res. Commun.,
1998
Oct
29
, 251 (806-10).
2043
Chouabe C
et al.
HERG and KvLQT1/IsK, the cardiac K+ channels involved in long QT syndromes, are targets for calcium channel blockers.
Mol. Pharmacol.,
1998
Oct
, 54 (695-703).
2044
Tinel N
et al.
The KCNQ2 potassium channel: splice variants, functional and developmental expression. Brain localization and comparison with KCNQ3.
FEBS Lett.,
1998
Nov
6
, 438 (171-6).
2045
Shimizu W
et al.
Cellular basis for the ECG features of the LQT1 form of the long-QT syndrome: effects of beta-adrenergic agonists and antagonists and sodium channel blockers on transmural dispersion of repolarization and torsade de pointes.
Circulation,
1998
Nov
24
, 98 (2314-22).
2046
Lo CF
et al.
Independent and exclusive modulation of cardiac delayed rectifying K+ current by protein kinase C and protein kinase A.
Circ. Res.,
1998
Nov
16
, 83 (995-1002).
2047
Jiang S
et al.
Strain-dependent developmental relaxation of imprinting of an endogenous mouse gene, Kvlqt1.
Genomics,
1998
Nov
1
, 53 (395-9).
2048
Tzounopoulos T
et al.
Gating of I(sK) channels expressed in Xenopus oocytes.
Biophys. J.,
1998
May
, 74 (2299-305).
2049
Demolombe S
et al.
A dominant negative isoform of the long QT syndrome 1 gene product.
J. Biol. Chem.,
1998
Mar
20
, 273 (6837-43).
2050
Gould TD
et al.
Imprinting of mouse Kvlqt1 is developmentally regulated.
Hum. Mol. Genet.,
1998
Mar
, 7 (483-7).
2051
Ackerman MJ
The long QT syndrome: ion channel diseases of the heart.
Mayo Clin. Proc.,
1998
Mar
, 73 (250-69).
2052
Satler CA
et al.
Multiple different missense mutations in the pore region of HERG in patients with long QT syndrome.
Hum. Genet.,
1998
Mar
, 102 (265-72).
2053
Nagai R
et al.
[Molecular genetics of cardiovascular diseases]
Rinsho Byori,
1998
Mar
, 46 (249-57).
2055
Aggeli A
et al.
Conformation and ion-channeling activity of a 27-residue peptide modeled on the single-transmembrane segment of the IsK (minK) protein.
Biochemistry,
1998
Jun
2
, 37 (8121-31).
2056
Kanters JK
et al.
Novel donor splice site mutation in the KVLQT1 gene is associated with long QT syndrome.
J. Cardiovasc. Electrophysiol.,
1998
Jun
, 9 (620-4).
2057
Caspary T
et al.
Multiple mechanisms regulate imprinting of the mouse distal chromosome 7 gene cluster.
Mol. Cell. Biol.,
1998
Jun
, 18 (3466-74).
2058
Yang WP
et al.
Functional expression of two KvLQT1-related potassium channels responsible for an inherited idiopathic epilepsy.
J. Biol. Chem.,
1998
Jul
31
, 273 (19419-23).
2059
An RH
et al.
Novel LQT-3 mutation affects Na+ channel activity through interactions between alpha- and beta1-subunits.
Circ. Res.,
1998
Jul
27
, 83 (141-6).
2060
Drici MD
et al.
Involvement of IsK-associated K+ channel in heart rate control of repolarization in a murine engineered model of Jervell and Lange-Nielsen syndrome.
Circ. Res.,
1998
Jul
13
, 83 (95-102).
2061
Tristani-Firouzi M
et al.
Voltage-dependent inactivation of the human K+ channel KvLQT1 is eliminated by association with minimal K+ channel (minK) subunits.
J. Physiol. (Lond.),
1998
Jul
1
, 510 ( Pt 1) (37-45).
2062
Splawski I
et al.
Genomic structure of three long QT syndrome genes: KVLQT1, HERG, and KCNE1.
Genomics,
1998
Jul
1
, 51 (86-97).
2063
Lipka LJ
et al.
Differential effects of bupivacaine on cardiac K channels: role of channel inactivation and subunit composition in drug-channel interaction.
J. Cardiovasc. Electrophysiol.,
1998
Jul
, 9 (727-42).
2065
Paulsen M
et al.
Syntenic organization of the mouse distal chromosome 7 imprinting cluster and the Beckwith-Wiedemann syndrome region in chromosome 11p15.5.
Hum. Mol. Genet.,
1998
Jul
, 7 (1149-59).
2066
Salata JJ
et al.
A novel benzodiazepine that activates cardiac slow delayed rectifier K+ currents.
Mol. Pharmacol.,
1998
Jul
, 54 (220-30).
2067
Duggal P
et al.
Mutation of the gene for IsK associated with both Jervell and Lange-Nielsen and Romano-Ward forms of Long-QT syndrome.
Circulation,
1998
Jan
20
, 97 (142-6).
2068
Marcus DC
et al.
Protein kinase C mediates P2U purinergic receptor inhibition of K+ channel in apical membrane of strial marginal cells.
Hear. Res.,
1998
Jan
, 115 (82-92).
2069
Charlier C
et al.
A pore mutation in a novel KQT-like potassium channel gene in an idiopathic epilepsy family.
Nat. Genet.,
1998
Jan
, 18 (53-5).
2070
Kambouris NG
et al.
Phenotypic characterization of a novel long-QT syndrome mutation (R1623Q) in the cardiac sodium channel.
Circulation,
1998
Feb
24
, 97 (640-4).
2071
Bianchi L
et al.
herg encodes a K+ current highly conserved in tumors of different histogenesis: a selective advantage for cancer cells?
Cancer Res.,
1998
Feb
15
, 58 (815-22).
2072
Makita N
et al.
A de novo missense mutation of human cardiac Na+ channel exhibiting novel molecular mechanisms of long QT syndrome.
FEBS Lett.,
1998
Feb
13
, 423 (5-9).
2073
Wang Q
et al.
Genetics, molecular mechanisms and management of long QT syndrome.
Ann. Med.,
1998
Feb
, 30 (58-65).
2074
Dumaine R
et al.
Mechanism of lidocaine block of late current in long Q-T mutant Na+ channels.
Am. J. Physiol.,
1998
Feb
, 274 (H477-87).
2075
Wang W
et al.
MinK-KvLQT1 fusion proteins, evidence for multiple stoichiometries of the assembled IsK channel.
J. Biol. Chem.,
1998
Dec
18
, 273 (34069-74).
2076
Pusch M
Increase of the single-channel conductance of KvLQT1 potassium channels induced by the association with minK.
Pflugers Arch.,
1998
Dec
, 437 (172-4).
2077
Sesti F
et al.
Single-channel characteristics of wild-type IKs channels and channels formed with two minK mutants that cause long QT syndrome.
J. Gen. Physiol.,
1998
Dec
, 112 (651-63).
2078
Yang Y
et al.
Single-channel properties of IKs potassium channels.
J. Gen. Physiol.,
1998
Dec
, 112 (665-78).
2079
Mall M
et al.
Cholinergic ion secretion in human colon requires coactivation by cAMP.
Am. J. Physiol.,
1998
Dec
, 275 (G1274-81).
2080
Wattanasirichaigoon D
et al.
Molecular genetics of long-QT syndrome.
Curr. Opin. Pediatr.,
1998
Dec
, 10 (628-34).
2081
Nakajima T
et al.
Novel mechanism of HERG current suppression in LQT2: shift in voltage dependence of HERG inactivation.
Circ. Res.,
1998
Aug
24
, 83 (415-22).
2082
Zhou Z
et al.
HERG channel dysfunction in human long QT syndrome. Intracellular transport and functional defects.
J. Biol. Chem.,
1998
Aug
14
, 273 (21061-6).
2083
Ackerman MJ
et al.
A novel mutation in KVLQT1 is the molecular basis of inherited long QT syndrome in a near-drowning patient's family.
Pediatr. Res.,
1998
Aug
, 44 (148-53).
2084
Pusch M
et al.
Activation and inactivation of homomeric KvLQT1 potassium channels.
Biophys. J.,
1998
Aug
, 75 (785-92).
2085
Li H
et al.
New mutations in the KVLQT1 potassium channel that cause long-QT syndrome.
Circulation,
1998
Apr
7
, 97 (1264-9).
2086
Shimizu W
et al.
Improvement of repolarization abnormalities by a K+ channel opener in the LQT1 form of congenital long-QT syndrome.
Circulation,
1998
Apr
28
, 97 (1581-8).
2087
Itoh T
et al.
Genomic organization and mutational analysis of HERG, a gene responsible for familial long QT syndrome.
Hum. Genet.,
1998
Apr
, 102 (435-9).
2088
Czajkowski V
et al.
[Long QT syndrome: from clinical discovery to molecular etiopathogenesis]
,
1998
Apr
, 53 (199-207).
2089
Vincent GM
The molecular genetics of the long QT syndrome: genes causing fainting and sudden death.
Annu. Rev. Med.,
1998
, 49 (263-74).
2090
Saarinen K
et al.
Molecular genetics of the long QT syndrome: two novel mutations of the KVLQT1 gene and phenotypic expression of the mutant gene in a large kindred.
Hum. Mutat.,
1998
, 11 (158-65).
2091
Nakamura M
et al.
KQT2, a new putative potassium channel family produced by alternative splicing. Isolation, genomic structure, and alternative splicing of the putative potassium channels.
Recept. Channels,
1998
, 5 (255-71).
2092
Benhorin J
et al.
Identification of a new SCN5A mutation, D1840G, associated with the long QT syndrome. Mutations in brief no. 153. Online.
Hum. Mutat.,
1998
, 12 (72).
2093
Couderc JP
et al.
Beat-to-beat repolarization variability in amplitude and duration in LQTS patients with the SCN5A sodium channel gene mutation.
,
1998
, 31 Suppl (134).
2094
Yamagishi H
et al.
A de novo missense mutation (R1623Q) of the SCN5A gene in a Japanese girl with sporadic long QT sydrome. Mutations in brief no. 140. Online.
Hum. Mutat.,
1998
, 11 (481).
2095
Shen CT
et al.
Multi-undulant T-U-wave, sinus bradycardia and long QT syndrome: a possible phenotype of mutant genes controlling the inward potassium rectifiers.
,
1997 Jul-Aug
, 38 (267-75).
2096
Jiang M
et al.
Suppression of slow delayed rectifier current by a truncated isoform of KvLQT1 cloned from normal human heart.
J. Biol. Chem.,
1997
Sep
26
, 272 (24109-12).
2097
Shalaby FY
et al.
Dominant-negative KvLQT1 mutations underlie the LQT1 form of long QT syndrome.
Circulation,
1997
Sep
16
, 96 (1733-6).
2098
Chouabe C
et al.
Properties of KvLQT1 K+ channel mutations in Romano-Ward and Jervell and Lange-Nielsen inherited cardiac arrhythmias.
EMBO J.,
1997
Sep
1
, 16 (5472-9).
2099
Heath BM
et al.
Block by propofol and thiopentone of the min K current (IsK) expressed in Xenopus oocytes.
Naunyn Schmiedebergs Arch. Pharmacol.,
1997
Sep
, 356 (404-9).
2100
Busch AE
et al.
The role of the IsK protein in the specific pharmacological properties of the IKs channel complex.
Br. J. Pharmacol.,
1997
Sep
, 122 (187-9).
2101
Splawski I
et al.
George M. Cober Lecturer: Mark T. Keating. Molecular basis of the long-QT syndrome associated with deafness.
Proc. Assoc. Am. Physicians,
1997
Sep
, 109 (504-11).
2102
van den Berg MH
et al.
The long QT syndrome: a novel missense mutation in the S6 region of the KVLQT1 gene.
Hum. Genet.,
1997
Sep
, 100 (356-61).
2103
Wollnik B
et al.
Pathophysiological mechanisms of dominant and recessive KVLQT1 K+ channel mutations found in inherited cardiac arrhythmias.
Hum. Mol. Genet.,
1997
Oct
, 6 (1943-9).
2104
Schulze-Bahr E
et al.
Autosomal recessive long-QT syndrome (Jervell Lange-Nielsen syndrome) is genetically heterogeneous.
Hum. Genet.,
1997
Oct
, 100 (573-6).
2105
Takimoto K
et al.
Decreased expression of Kv4.2 and novel Kv4.3 K+ channel subunit mRNAs in ventricles of renovascular hypertensive rats.
Circ. Res.,
1997
Oct
, 81 (533-9).
2106
Donger C
et al.
KVLQT1 C-terminal missense mutation causes a forme fruste long-QT syndrome.
Circulation,
1997
Nov
4
, 96 (2778-81).
2107
Morey TE
et al.
Ionic basis of the differential effects of intravenous anesthetics on erythromycin-induced prolongation of ventricular repolarization in the guinea pig heart.
Anesthesiology,
1997
Nov
, 87 (1172-81).
2108
Splawski I
et al.
Mutations in the hminK gene cause long QT syndrome and suppress IKs function.
Nat. Genet.,
1997
Nov
, 17 (338-40).
2109
London B
et al.
Two isoforms of the mouse ether-a-go-go-related gene coassemble to form channels with properties similar to the rapidly activating component of the cardiac delayed rectifier K+ current.
Circ. Res.,
1997
Nov
, 81 (870-8).
2110
Tyson J
et al.
IsK and KvLQT1: mutation in either of the two subunits of the slow component of the delayed rectifier potassium channel can cause Jervell and Lange-Nielsen syndrome.
Hum. Mol. Genet.,
1997
Nov
, 6 (2179-85).
2111
Wang Q
et al.
Molecular genetics of long QT syndrome from genes to patients.
Curr. Opin. Cardiol.,
1997
May
, 12 (310-20).
2112
Chiesa N
et al.
A novel role for HERG K+ channels: spike-frequency adaptation.
J. Physiol. (Lond.),
1997
Jun
1
, 501 ( Pt 2) (313-8).
2113
Le Marec H
et al.
[Congenital long QT syndromes]
Arch Mal Coeur Vaiss,
1997
Jun
, 90 Spec No 3 (25-35).
2114
Romey G
et al.
Molecular mechanism and functional significance of the MinK control of the KvLQT1 channel activity.
J. Biol. Chem.,
1997
Jul
4
, 272 (16713-6).
2115
McDonald TV
et al.
A minK-HERG complex regulates the cardiac potassium current I(Kr).
Nature,
1997
Jul
17
, 388 (289-92).
2116
Kaczmarek LK
et al.
Properties and regulation of the minK potassium channel protein.
Physiol. Rev.,
1997
Jul
, 77 (627-41).
2117
Busch AE
et al.
Role of the ISK protein in the IminK channel complex.
Trends Pharmacol. Sci.,
1997
Jan
, 18 (26-9).
2118
Tanaka T
et al.
Four novel KVLQT1 and four novel HERG mutations in familial long-QT syndrome.
Circulation,
1997
Feb
4
, 95 (565-7).
2119
Brahmajothi MV
et al.
Heterogeneity in K+ channel transcript expression detected in isolated ferret cardiac myocytes.
,
1997
Feb
, 20 (388-96).
2121
Lee MP
et al.
Human KVLQT1 gene shows tissue-specific imprinting and encompasses Beckwith-Wiedemann syndrome chromosomal rearrangements.
Nat. Genet.,
1997
Feb
, 15 (181-5).
2122
Neyroud N
et al.
A novel mutation in the potassium channel gene KVLQT1 causes the Jervell and Lange-Nielsen cardioauditory syndrome.
Nat. Genet.,
1997
Feb
, 15 (186-9).
2123
Shi W
et al.
Identification of two nervous system-specific members of the erg potassium channel gene family.
J. Neurosci.,
1997
Dec
15
, 17 (9423-32).
2124
Loussouarn G
et al.
KvLQT1 potassium channel but not IsK is the molecular target for trans-6-cyano-4-(N-ethylsulfonyl-N-methylamino)-3-hydroxy-2,2-dimethyl- chromane.
Mol. Pharmacol.,
1997
Dec
, 52 (1131-6).
2125
Marcus DC
et al.
P2U purinergic receptor inhibits apical IsK/KvLQT1 channel via protein kinase C in vestibular dark cells.
Am. J. Physiol.,
1997
Dec
, 273 (C2022-9).
2126
Sunose H
et al.
cAMP increases K+ secretion via activation of apical IsK/KvLQT1 channels in strial marginal cells.
Hear. Res.,
1997
Dec
, 114 (107-16).
2127
Street VA
et al.
Physical mapping of potassium channel gene clusters on mouse chromosomes three and six.
Genomics,
1997
Aug
15
, 44 (110-7).
2128
Lee MP
et al.
Low frequency of p57KIP2 mutation in Beckwith-Wiedemann syndrome.
Am. J. Hum. Genet.,
1997
Aug
, 61 (304-9).
2129
Moss AJ
Clinical management of patients with the long QT syndrome: drugs, devices, and gene-specific therapy.
,
1997
Aug
, 20 (2058-60).
2130
Priori SG
et al.
Molecular biology of the long QT syndrome: impact on management.
,
1997
Aug
, 20 (2052-7).
2131
Kimura T
et al.
[A family with facioscapulohumeral muscular dystrophy and hereditary long QT syndrome]
Rinsho Shinkeigaku,
1997
Aug
, 37 (690-2).
2132
Bleich M
et al.
KVLQT channels are inhibited by the K+ channel blocker 293B.
Pflugers Arch.,
1997
Aug
, 434 (499-501).
2133
Yang WP
et al.
KvLQT1, a voltage-gated potassium channel responsible for human cardiac arrhythmias.
Proc. Natl. Acad. Sci. U.S.A.,
1997
Apr
15
, 94 (4017-21).
2134
Wang DW
et al.
Pharmacological targeting of long QT mutant sodium channels.
J. Clin. Invest.,
1997
Apr
1
, 99 (1714-20).
2135
Sanguinetti MC
et al.
Molecular physiology of cardiac delayed rectifier K+ channels.
,
1997
, Suppl 12 (170-2).
2136
Lucaci L
[The electrophysiological mechanism of the long QT syndrome]
,
1996 Jul-Dec
, 100 (32-6).
2137
Priori SG
et al.
A molecular basis for the therapy of the long QT syndrome.
Arch Mal Coeur Vaiss,
1996
Sep
, 89 (1185-7).
2138
Satler CA
et al.
Novel missense mutation in the cyclic nucleotide-binding domain of HERG causes long QT syndrome.
Am. J. Med. Genet.,
1996
Oct
2
, 65 (27-35).
2139
Roden DM
et al.
Multiple mechanisms in the long-QT syndrome. Current knowledge, gaps, and future directions. The SADS Foundation Task Force on LQTS.
Circulation,
1996
Oct
15
, 94 (1996-2012).
2140
Priori SG
et al.
The long QT syndrome: new diagnostic and therapeutic approach in the era of molecular biology.
,
1996
Oct
12
, 126 (1727-31).
2141
Barhanin J
et al.
K(V)LQT1 and lsK (minK) proteins associate to form the I(Ks) cardiac potassium current.
Nature,
1996
Nov
7
, 384 (78-80).
2142
Sanguinetti MC
et al.
Coassembly of K(V)LQT1 and minK (IsK) proteins to form cardiac I(Ks) potassium channel.
Nature,
1996
Nov
7
, 384 (80-3).
2143
Wang DW
et al.
Characterization of human cardiac Na+ channel mutations in the congenital long QT syndrome.
Proc. Natl. Acad. Sci. U.S.A.,
1996
Nov
12
, 93 (13200-5).
2144
Wang Q
et al.
Genomic organization of the human SCN5A gene encoding the cardiac sodium channel.
Genomics,
1996
May
15
, 34 (9-16).
2145
Benson DW
et al.
Missense mutation in the pore region of HERG causes familial long QT syndrome.
Circulation,
1996
May
15
, 93 (1791-5).
2146
Dumaine R
et al.
Multiple mechanisms of Na+ channel--linked long-QT syndrome.
Circ. Res.,
1996
May
, 78 (916-24).
2147
Sanguinetti MC
et al.
Spectrum of HERG K+-channel dysfunction in an inherited cardiac arrhythmia.
Proc. Natl. Acad. Sci. U.S.A.,
1996
Mar
5
, 93 (2208-12).
2149
Brahmajothi MV
et al.
In situ hybridization reveals extensive diversity of K+ channel mRNA in isolated ferret cardiac myocytes.
Circ. Res.,
1996
Jun
, 78 (1083-9).
2150
Priori SG
et al.
Differential response to Na+ channel blockade, beta-adrenergic stimulation, and rapid pacing in a cellular model mimicking the SCN5A and HERG defects present in the long-QT syndrome.
Circ. Res.,
1996
Jun
, 78 (1009-15).
2151
An RH
et al.
Lidocaine block of LQT-3 mutant human Na+ channels.
Circ. Res.,
1996
Jul
, 79 (103-8).
2152
Wang Q
et al.
Positional cloning of a novel potassium channel gene: KVLQT1 mutations cause cardiac arrhythmias.
Nat. Genet.,
1996
Jan
, 12 (17-23).
2153
Wang Q
et al.
Cardiac sodium channel mutations in patients with long QT syndrome, an inherited cardiac arrhythmia.
Hum. Mol. Genet.,
1995
Sep
, 4 (1603-7).
2154
Wang Q
et al.
SCN5A mutations associated with an inherited cardiac arrhythmia, long QT syndrome.
Cell,
1995
Mar
10
, 80 (805-11).
2155
Curran ME
et al.
A molecular basis for cardiac arrhythmia: HERG mutations cause long QT syndrome.
Cell,
1995
Mar
10
, 80 (795-803).
2156
Keating MT
Genetic approaches to cardiovascular disease. Supravalvular aortic stenosis, Williams syndrome, and long-QT syndrome.
Circulation,
1995
Jul
1
, 92 (142-7).
2157
Schwartz PJ
et al.
Long QT syndrome patients with mutations of the SCN5A and HERG genes have differential responses to Na+ channel blockade and to increases in heart rate. Implications for gene-specific therapy.
Circulation,
1995
Dec
15
, 92 (3381-6).
2158
Bennett PB
et al.
Molecular mechanism for an inherited cardiac arrhythmia.
Nature,
1995
Aug
24
, 376 (683-5).
2159
Sanguinetti MC
et al.
A mechanistic link between an inherited and an acquired cardiac arrhythmia: HERG encodes the IKr potassium channel.
Cell,
1995
Apr
21
, 81 (299-307).
2160
De Ferrari GM
et al.
Effect of calcium channel block on the wall motion abnormality of the idiopathic long QT syndrome.
Circulation,
1994
May
, 89 (2126-32).
2161
Krause PC
et al.
Characteristics of Ca(2+)-activated K+ channels isolated from the left ventricle of a patient with idiopathic long QT syndrome.
Am. Heart J.,
1993
Nov
, 126 (1134-41).
2162
Tejedor FJ
et al.
Photoaffinity labeling of the receptor site for alpha-scorpion toxins on purified and reconstituted sodium channels by a new toxin derivative.
Cell. Mol. Neurobiol.,
1990
Jun
, 10 (257-65).
2163
Tejedor FJ
et al.
Site of covalent attachment of alpha-scorpion toxin derivatives in domain I of the sodium channel alpha subunit.
Proc. Natl. Acad. Sci. U.S.A.,
1988
Nov
, 85 (8742-6).
2164
Warashina A
et al.
Binding properties of sea anemone toxins to sodium channels in the crayfish giant axon.
Comp. Biochem. Physiol. C, Comp. Pharmacol. Toxicol.,
1988
, 90 (351-9).
2165
Sharkey RG
et al.
Allosteric modulation of neurotoxin binding to voltage-sensitive sodium channels by Ptychodiscus brevis toxin 2.
Mol. Pharmacol.,
1987
Mar
, 31 (273-8).
2166
Messner DJ
et al.
Functional properties of rat brain sodium channels lacking the beta 1 or beta 2 subunit.
J. Biol. Chem.,
1986
Nov
15
, 261 (14882-90).
2167
Feller DJ
et al.
The sodium channel from rat brain. Reconstitution of voltage-dependent scorpion toxin binding in vesicles of defined lipid composition.
J. Biol. Chem.,
1985
Sep
25
, 260 (11542-7).
2168
Tamkun MM
et al.
The sodium channel from rat brain. Reconstitution of neurotoxin-activated ion flux and scorpion toxin binding from purified components.
J. Biol. Chem.,
1984
Feb
10
, 259 (1676-88).