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

Wu W et al. Molecular Basis of Cardiac Delayed Rectifier Potassium Channel Function and Pharmacology.
Card Electrophysiol Clin, 2016 Jun , 8 (275-84).

8

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).

9

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).

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

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 , ().

17

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).

18

Ichikawa M et al. Multigenerational Inheritance of Long QT Syndrome Type 2 in a Japanese Family.
Intern. Med., 2016 , 55 (259-62).

19

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 ().

20

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).

21

Choi JI et al. α1-Syntrophin Variant Identified in Drug-Induced Long QT Syndrome Increases Late Sodium Current.
PLoS ONE, 2016 , 11 (e0152355).

22

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).

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

Powell KL et al. In response: LQT, HCN, and epilepsy model.
Epilepsia, 2015 Nov , 56 (1855-6).

37

Kodirov SA LQT, HCN, and epilepsy model.
Epilepsia, 2015 Nov , 56 (1855).

38

Vassilakopoulou V et al. Distinctive malfunctions of calmodulin mutations associated with heart RyR2-mediated arrhythmic disease.
Biochim. Biophys. Acta, 2015 Nov , 1850 (2168-76).

39

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).

40

Zoledziewska M et al. Height-reducing variants and selection for short stature in Sardinia.
Nat. Genet., 2015 Nov , 47 (1352-6).

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

Jahangir A et al. Strain Echocardiography and LQTS Subtypes: Mechanical Alterations in an Electrical Disorder.
JACC Cardiovasc Imaging, 2015 May , 8 (511-3).

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

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).

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

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).

53

Laurentino S et al. Epigenetic germline mosaicism in infertile men.
Hum. Mol. Genet., 2015 Mar 1 , 24 (1295-304).

54

Stott JB et al. Contribution of kv7 channels to natriuretic Peptide mediated vasodilation in normal and hypertensive rats.
Hypertension, 2015 Mar , 65 (676-82).

55

Yang L et al. Intravenous anesthetic propofol inhibits multiple human cardiac potassium channels.
Anesthesiology, 2015 Mar , 122 (571-84).

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

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).

59

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).

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

Mills TA et al. Activation of KV7 channels stimulates vasodilatation of human placental chorionic plate arteries.
Placenta, 2015 Jun , 36 (638-44).

63

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).

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

Issa NP et al. QT interval prolongation in a patient with LQT2 on levetiracetam.
Seizure, 2015 Jul , 29 (134-6).

69

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).

70

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).

71

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).

72

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).

73

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).

74

Kohl B et al. Solid phase synthesis, NMR structure determination of α-KTx3.8, its in silico docking to Kv1.x potassium channels, and electrophysiological analysis provide insights into toxin-channel selectivity.
Toxicon, 2015 Jul , 101 (70-8).

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

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).

84

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).

85

Chen M et al. Pharmacogenomics of glinides.
Pharmacogenomics, 2015 Jan , 16 (45-60).

86

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).

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

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).

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

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).

92

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).

93

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).

94

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).

95

Lee S et al. Heterogeneity in Kv7 channel function in the cerebral and coronary circulation.
Microcirculation, 2015 Feb , 22 (109-21).

96

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).

97

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).

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

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).

103

Qureshi SF et al. Mutational analysis of SCN5A gene in long QT syndrome.
Meta Gene, 2015 Dec , 6 (26-35).

104

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).

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

Lampert R Reassuring News for Genetically Tested, Appropriately Treated, Low-Risk LQTS Patients.
J. Cardiovasc. Electrophysiol., 2015 Aug , 26 (859-61).

110

Krönauer T et al. [Perioperative treatment of patients with long QT syndrome].
Anaesthesist, 2015 Aug , 64 (625-39).

111

Krönauer T et al. [Long QT syndrome : History, genetics, clinical symptoms, causes and therapy].
Anaesthesist, 2015 Aug , 64 (586-95).

112

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).

113

Li G et al. RNA interference-based therapeutics for inherited long QT syndrome.
Exp Ther Med, 2015 Aug , 10 (395-400).

114

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).

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

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).

117

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).

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

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).

127

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).

128

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).

129

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).

130

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).

131

Lazzerini PE et al. Long QT Syndrome: An Emerging Role for Inflammation and Immunity.
Front Cardiovasc Med, 2015 , 2 (26).

132

Warsi J et al. Regulation of Voltage Gated K+ Channel KCNE1/KCNQ1 by the Janus Kinase JAK3.
Cell. Physiol. Biochem., 2015 , 37 (2476-85).

133

Cazals Y et al. KCNK5 channels mostly expressed in cochlear outer sulcus cells are indispensable for hearing.
Nat Commun, 2015 , 6 (8780).

134

Bellin M et al. Human iPS cell models of Jervell and Lange-Nielsen syndrome.
Rare Dis, 2015 , 3 (e1012978).

135

Huerta-Chagoya A et al. Genetic determinants for gestational diabetes mellitus and related metabolic traits in Mexican women.
PLoS ONE, 2015 , 10 (e0126408).

136

Hu RM et al. Arrhythmogenic Biophysical Phenotype for SCN5A Mutation S1787N Depends upon Splice Variant Background and Intracellular Acidosis.
PLoS ONE, 2015 , 10 (e0124921).

137

Liu QN et al. Eag Domains Regulate LQT Mutant hERG Channels in Human Induced Pluripotent Stem Cell-Derived Cardiomyocytes.
PLoS ONE, 2015 , 10 (e0123951).

138

Ma D et al. Characterization of a novel KCNQ1 mutation for type 1 long QT syndrome and assessment of the therapeutic potential of a novel IKs activator using patient-specific induced pluripotent stem cell-derived cardiomyocytes.
Stem Cell Res Ther, 2015 , 6 (39).

139

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).

140

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).

141

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).

142

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).

143

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).

144

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).

145

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).

146

Itoh H et al. A Common Mutation of Long QT Syndrome Type 1 in Japan.
Circ. J., 2015 , 79 (2026-30).

147

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).

148

Mayo S et al. In Pursuit of New Imprinting Syndromes by Epimutation Screening in Idiopathic Neurodevelopmental Disorder Patients.
Biomed Res Int, 2015 , 2015 (341986).

149

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).

150

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).

151

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).

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

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).

154

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).

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

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).

158

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).

159

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).

160

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).

161

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).

162

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).

163

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).

164

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).

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

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).

171

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).

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

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).

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

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).

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

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).

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

Ki CS et al. A KCNQ1 mutation causes age-dependant bradycardia and persistent atrial fibrillation.
Pflugers Arch., 2014 Mar , 466 (529-40).

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

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).

201

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).

202

Liu W et al. KCNE2 modulates cardiac L-type Ca(2+) channel.
J. Mol. Cell. Cardiol., 2014 Jul , 72 (208-18).

203

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).

204

Glengarry JM et al. Long QT molecular autopsy in sudden infant death syndrome.
Arch. Dis. Child., 2014 Jul , 99 (635-40).

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

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).

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

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).

212

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).

213

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).

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

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).

217

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).

218

Zhang Y et al. The SCN5A mutation A1180V is associated with electrocardiographic features of LQT3.
Pediatr Cardiol, 2014 Feb , 35 (295-300).

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

Bond R et al. Congenital long QT syndrome: a case report of LQT2 and LQT13 in a neonate.
Europace, 2014 Dec , 16 (1807).

224

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).

225

Salsbury G et al. Disruption of the potassium channel regulatory subunit KCNE2 causes iron-deficient anemia.
Exp. Hematol., 2014 Dec , 42 (1053-8.e1).

226

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).

227

Fukuyama M et al. Long QT syndrome type 8: novel CACNA1C mutations causing QT prolongation and variant phenotypes.
Europace, 2014 Dec , 16 (1828-37).

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

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).

233

Imai M et al. Novel KCNQ1 splicing mutation in patients with forme fruste LQT1 aggravated by hypokalemia.
J Cardiol, 2014 Aug , 64 (121-6).

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

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).

238

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).

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

Wang D et al. Cardiac channelopathy testing in 274 ethnically diverse sudden unexplained deaths.
Forensic Sci. Int., 2014 Apr , 237 (90-9).

241

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).

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

Dvir M et al. Recent molecular insights from mutated IKS channels in cardiac arrhythmia.
Curr Opin Pharmacol, 2014 Apr , 15 (74-82).

244

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).

245

Torekov SS et al. KCNQ1 long QT syndrome patients have hyperinsulinemia and symptomatic hypoglycemia.
Diabetes, 2014 Apr , 63 (1315-25).

246

Tester DJ et al. GENETICS OF LONG QT SYNDROME.
Methodist Debakey Cardiovasc J, 2014 1 , 10 (29-33).

247

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).

248

Strutz-Seebohm N et al. Klotho: a new trafficking modifier of Kv7.1/KCNE1 channels.
Channels (Austin), 2014 , 8 (285).

249

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).

250

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).

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

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).

253

Zaydman MA et al. Domain-domain interactions determine the gating, permeation, pharmacology, and subunit modulation of the IKs ion channel.
Elife, 2014 , 3 (e03606).

254

Shimizu W Clinical and genetic diagnosis for inherited cardiac arrhythmias.
J Nippon Med Sch, 2014 , 81 (203-10).

255

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).

256

Biernacka EK [Genetic arrhythmias and gender].
Prz. Lek., 2014 , 71 (139-41).

257

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).

258

Miller D et al. Sodium channels, cardiac arrhythmia, and therapeutic strategy.
Adv. Pharmacol., 2014 , 70 (367-92).

259

Li P et al. Differential modulations of KCNQ1 by auxiliary proteins KCNE1 and KCNE2.
Sci Rep, 2014 , 4 (4973).

260

Villafane J et al. Short QT syndrome manifesting with neonatal atrial fibrillation and bradycardia.
Cardiology, 2014 , 128 (236-40).

261

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).

262

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).

263

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).

264

Barro-Soria R et al. KCNE1 divides the voltage sensor movement in KCNQ1/KCNE1 channels into two steps.
Nat Commun, 2014 , 5 (3750).

265

Antzelevitch C et al. The role of late I Na in development of cardiac arrhythmias.
Handb Exp Pharmacol, 2014 , 221 (137-68).

266

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).

267

Wu C et al. Kcnq1-5 (Kv7.1-5) potassium channel expression in the adult zebrafish.
BMC Physiol., 2014 , 14 (1).

268

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).

269

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).

270

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).

271

Zimmer T et al. Voltage-gated sodium channels in the mammalian heart.
Glob Cardiol Sci Pract, 2014 , 2014 (449-63).

272

Guo X et al. Discovery of aroyl piperazine derivatives as IKr & IKs dual inhibitors for cardiac arrhythmia treatment.
Med Chem, 2014 , 10 (497-505).

273

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).

274

Jimmy JJ et al. Clinical characteristics of patients with congenital long QT syndrome and bigenic mutations.
Chin. Med. J., 2014 , 127 (1482-6).

275

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).

276

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).

277

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).

278

Mizusawa Y et al. Genetic and clinical advances in congenital long QT syndrome.
Circ. J., 2014 , 78 (2827-33).

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

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).

281

Coyan FC et al. A long QT mutation substitutes cholesterol for phosphatidylinositol-4,5-bisphosphate in KCNQ1 channel regulation.
PLoS ONE, 2014 , 9 (e93255).

282

Almilaji A et al. Upregulation of KCNQ1/KCNE1 K+ channels by Klotho.
Channels (Austin), 2014 , 8 (222-9).

283

Ertugrul I et al. Follow up of a family with asymptomatic compound long QT syndrome mutations.
Genet. Couns., 2014 , 25 (399-403).

284

Fan Z et al. Efficient gene targeting in golden Syrian hamsters by the CRISPR/Cas9 system.
PLoS ONE, 2014 , 9 (e109755).

285

Nakajo K et al. Steric hindrance between S4 and S5 of the KCNQ1/KCNE1 channel hampers pore opening.
Nat Commun, 2014 , 5 (4100).

286

Qureshi SF et al. Novel mutations of KCNQ1 in Long QT syndrome.
Indian Heart J, 2013 Sep-Oct , 65 (552-60).

287

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).

288

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).

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

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).

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

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).

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

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 , ().

305

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).

306

Meng J et al. Compound ICA-105574 prevents arrhythmias induced by cardiac delayed repolarization.
Eur. J. Pharmacol., 2013 Oct 15 , 718 (87-97).

307

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).

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

Giudicessi JR et al. Genotype- and Phenotype-Guided Management of Congenital Long QT Syndrome.
Curr Probl Cardiol, 2013 Oct , 38 (417-455).

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

Campbell CM et al. Selective targeting of gain-of-function KCNQ1 mutations predisposing to atrial fibrillation.
Circ Arrhythm Electrophysiol, 2013 Oct , 6 (960-6).

312

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).

313

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).

314

Lee YS et al. Long QT syndrome: a Korean single center study.
J. Korean Med. Sci., 2013 Oct , 28 (1454-60).

315

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).

316

Yu W et al. Association between KCNQ1 genetic variants and QT interval in a Chinese population.
Diabet. Med., 2013 Oct , 30 (1225-9).

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

Zhang X et al. KCNQ and KCNE potassium channel subunit expression in bovine retinal pigment epithelium.
Exp. Eye Res., 2013 Nov , 116 (424-32).

327

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).

328

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).

329

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).

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

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).

333

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).

334

Bartos DC et al. A KCNQ1 mutation causes a high penetrance for familial atrial fibrillation.
J. Cardiovasc. Electrophysiol., 2013 May , 24 (562-9).

335

Hoosien M et al. Dysfunctional potassium channel subunit interaction as a novel mechanism of long QT syndrome.
Heart Rhythm, 2013 May , 10 (728-37).

336

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).

337

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).

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

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).

344

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).

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

Heijman J et al. KCNQ1 autoantibodies: another way to regulate IKs.
Cardiovasc. Res., 2013 Jun 1 , 98 (329-31).

349

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).

350

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).

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

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).

353

Lang F et al. Therapeutic potential of serum and glucocorticoid inducible kinase inhibition.
Expert Opin Investig Drugs, 2013 Jun , 22 (701-14).

354

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).

355

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).

356

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).

357

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).

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

Jost N et al. Ionic mechanisms limiting cardiac repolarization-reserve in humans compared to dogs.
J. Physiol. (Lond.), 2013 Jul 22 , ().

361

Amin AS et al. Long QT syndrome: beyond the causal mutation.
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

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).

374

Abbott GW KCNE genetics and pharmacogenomics in cardiac arrhythmias: much ado about nothing?
Expert Rev Clin Pharmacol, 2013 Jan , 6 (49-60).

375

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).

376

Lang F et al. Regulation of ion channels by the serum- and glucocorticoid-inducible kinase SGK1.
FASEB J., 2013 Jan , 27 (3-12).

377

Giudicessi JR et al. Determinants of incomplete penetrance and variable expressivity in heritable cardiac arrhythmia syndromes.
Transl Res, 2013 Jan , 161 (1-14).

378

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).

379

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).

380

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).

381

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).

382

Feng MJ et al. [KCNQ1 mutation in patients with lone atrial fibrillation].
Zhonghua Xin Xue Guan Bing Za Zhi, 2013 Jan , 41 (8-12).

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

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).

388

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).

389

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).

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

Seebohm G A complex partnership: KCNQ1 and KCNE1.
Biophys. J., 2013 Dec 3 , 105 (2437-8).

393

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).

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

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).

405

Thomsen MB Kv7.1 isoform gradients in the heart: new potential approach to alter repolarization reserve.
Heart Rhythm, 2013 Aug , 10 (1229-30).

406

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).

407

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).

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

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).

410

Lee HC et al. Modulation of KCNQ1 alternative splicing regulates cardiac IKs and action potential repolarization.
Heart Rhythm, 2013 Aug , 10 (1220-8).

411

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).

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

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).

418

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).

419

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).

420

Hsiao PY et al. Gene mutations in cardiac arrhythmias: a review of recent evidence in ion channelopathies.
Appl Clin Genet, 2013 , 6 (1-13).

421

Li X et al. Endolymphatic Na(+) and K(+) Concentrations during Cochlear Growth and Enlargement in Mice Lacking Slc26a4/pendrin.
PLoS ONE, 2013 , 8 (e65977).

422

Hua Z et al. Chemical derivatization and purification of peptide-toxins for probing ion channel complexes.
Methods Mol. Biol., 2013 , 995 (19-30).

423

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).

424

Moric-Janiszewska E et al. Quantitative PCR as an Alternative in the Diagnosis of Long-QT Syndrome.
Biomed Res Int, 2013 , 2013 (418604).

425

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).

426

Fan Y et al. Genistein ameliorates adverse cardiac effects induced by arsenic trioxide through preventing cardiomyocytes apoptosis.
Cell. Physiol. Biochem., 2013 , 31 (80-91).

427

Shimizu W Update of diagnosis and management of inherited cardiac arrhythmias.
Circ. J., 2013 , 77 (2867-72).

428

Netchine I et al. Epigenetic anomalies in childhood growth disorders.
Nestle Nutr Inst Workshop Ser, 2013 , 71 (65-73).

429

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).

430

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).

431

Trolle C et al. Long QT interval in Turner syndrome--a high prevalence of LQTS gene mutations.
PLoS ONE, 2013 , 8 (e69614).

432

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).

433

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).

434

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).

435

Ferrer T et al. Tamoxifen inhibition of kv7.2/kv7.3 channels.
PLoS ONE, 2013 , 8 (e76085).

436

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).

437

Pérez Riera AR et al. Congenital short QT syndrome: landmarks of the newest arrhythmogenic cardiac channelopathy.
Cardiol J, 2013 , 20 (464-71).

438

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).

439

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).

440

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).

441

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).

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

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).

445

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).

446

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).

447

Zumhagen S et al. Inherited long QT syndrome: clinical manifestation, genetic diagnostics, and therapy.
Herzschrittmacherther Elektrophysiol, 2012 Sep , 23 (211-9).

448

Lim XL et al. KCNQ1 SNPS and susceptibility to diabetic nephropathy in East Asians with type 2 diabetes.
Diabetologia, 2012 Sep , 55 (2402-6).

449

Wijnen M et al. KCNQ1OT1 hypomethylation: a novel disguised genetic predisposition in sporadic pediatric adrenocortical tumors?
Pediatr Blood Cancer, 2012 Sep , 59 (565-6).

450

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).

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

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).

457

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).

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

Mannino GC et al. Individualized therapy for type 2 diabetes: clinical implications of pharmacogenetic data.
Mol Diagn Ther, 2012 Oct , 16 (285-302).

460

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).

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

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).

466

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).

467

Edelmann J et al. Long QT syndrome mutation detection by SNaPshot technique.
Int. J. Legal Med., 2012 Nov , 126 (969-73).

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 , ().

471

Wilmes J et al. Regulation of KCNQ1/KCNE1 by β-catenin.
, 2012 May 14 , ().

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

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).

475

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).

476

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).

477

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).

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

Barsheshet A et al. Mutations in Cytoplasmic Loops of the KCNQ1 Channel and the Risk of Life-Threatening Events: Implications for Mutation-Specific Response to Beta-Blocker Therapy in Type-1 Long QT Syndrome.
, 2012 Mar 28 , ().

481

Onay OS et al. Successful Implantation of an Intracardiac Defibrillator in an Infant With Long QT Syndrome and Isolated Noncompaction of the Ventricular Myocardium.
, 2012 Mar 23 , ().

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

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).

485

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).

486

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).

487

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).

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

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).

493

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).

494

Li Y et al. The A-kinase anchoring protein Yotiao facilitates complex formation between type 9 adenylyl cyclase and the IKs potassium channel in heart.
, 2012 Jul 9 , ().

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

Vandenberg JI et al. hERG K(+) channels: structure, function, and clinical significance.
Physiol. Rev., 2012 Jul , 92 (1393-478).

503

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).

504

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).

505

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).

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

Krishnan Y et al. Partially dominant mutant channel defect corresponding with intermediate LQT2 phenotype.
Pacing Clin Electrophysiol, 2012 Jan , 35 (3-16).

513

Andersen MN et al. AMP-activated protein kinase downregulates Kv7.1 cell surface expression.
Traffic, 2012 Jan , 13 (143-56).

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

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).

519

Dennis AT et al. Molecular determinants of pentamidine-induced hERG trafficking inhibition.
Mol. Pharmacol., 2012 Feb , 81 (198-209).

520

Chan PJ et al. Characterization of KCNQ1 atrial fibrillation mutations reveals distinct dependence on KCNE1.
J. Gen. Physiol., 2012 Feb , 139 (135-44).

521

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).

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

Friedrichs S et al. Modeling long QT syndromes using induced pluripotent stem cells: Current progress and future challenges.
Trends Cardiovasc. Med., 2012 Dec 18 , ().

526

Ishii K et al. Endocytic regulation of voltage-dependent potassium channels in the heart.
J. Pharmacol. Sci., 2012 Dec 18 , 120 (264-9).

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

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).

532

Fedoriw AM et al. Differentiation-driven nucleolar association of the mouse imprinted Kcnq1 locus.
G3 (Bethesda), 2012 Dec , 2 (1521-8).

533

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).

534

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).

535

Wang Y et al. Probing the structural basis for differential KCNQ1 modulation by KCNE1 and KCNE2.
J. Gen. Physiol., 2012 Dec , 140 (653-69).

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

Mohammad F et al. Long noncoding RNA-mediated maintenance of DNA methylation and transcriptional gene silencing.
Development, 2012 Aug , 139 (2792-803).

538

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).

539

Ware JS et al. Paralogous annotation of disease-causing variants in long QT syndrome genes.
Hum. Mutat., 2012 Aug , 33 (1188-91).

540

Purtell K et al. The KCNQ1-KCNE2 K⁺ channel is required for adequate thyroid I⁻ uptake.
FASEB J., 2012 Aug , 26 (3252-9).

541

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).

542

Song W et al. Cardiac sodium channel Nav1.5 mutations and cardiac arrhythmia.
Pediatr Cardiol, 2012 Aug , 33 (943-9).

543

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).

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

Komarlu R et al. Fetal and neonatal presentation of long QT syndrome.
Pacing Clin Electrophysiol, 2012 Apr , 35 (e87-90).

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

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).

552

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).

553

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).

554

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).

555

Wang Y et al. Trafficking-deficient G572R-hERG and E637K-hERG activate stress and clearance pathways in endoplasmic reticulum.
PLoS ONE, 2012 , 7 (e29885).

556

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).

557

Morris LM et al. Mouse middle ear ion homeostasis channels and intercellular junctions.
PLoS ONE, 2012 , 7 (e39004).

558

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).

559

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).

560

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).

561

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).

562

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).

563

Knoche JW et al. Atrial Fibrillation and Long QT Syndrome Presenting in a 12-Year-Old Girl.
Case Rep Pediatr, 2012 , 2012 (124838).

564

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).

565

Hoekstra M et al. Induced pluripotent stem cell derived cardiomyocytes as models for cardiac arrhythmias.
Front Physiol, 2012 , 3 (346).

566

Stump MR et al. Isoform-specific dominant-negative effects associated with hERG1 G628S mutation in long QT syndrome.
PLoS ONE, 2012 , 7 (e42552).

567

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).

568

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).

569

Chen ZY et al. Structural and functional diversity of acidic scorpion potassium channel toxins.
PLoS ONE, 2012 , 7 (e35154).

570

Chen XD et al. Several polymorphisms of KCNQ1 gene are associated with plasma lipid levels in general Chinese populations.
PLoS ONE, 2012 , 7 (e34229).

571

Sato A et al. Long QT syndrome with nocturnal cardiac events caused by a KCNH2 missense mutation (G604S).
Intern. Med., 2012 , 51 (1857-60).

572

Xu XH et al. Allitridi inhibits multiple cardiac potassium channels expressed in HEK 293 cells.
PLoS ONE, 2012 , 7 (e51550).

573

Lazarczyk MJ et al. Selective acquired long QT syndrome (saLQTS) upon risperidone treatment.
BMC Psychiatry, 2012 , 12 (220).

574

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).

575

Sun Q et al. The association between KCNQ1 gene polymorphism and type 2 diabetes risk: a meta-analysis.
PLoS ONE, 2012 , 7 (e48578).

576

Adalsteinsson BT et al. Heterogeneity in white blood cells has potential to confound DNA methylation measurements.
PLoS ONE, 2012 , 7 (e46705).

577

Ferro F et al. Long-chain acylcarnitines regulate the hERG channel.
PLoS ONE, 2012 , 7 (e41686).

578

Moric-Janiszewska E et al. Molecular diagnostics of families with long-QT syndrome.
Cardiol J, 2012 , 19 (159-67).

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

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).

584

Dennis AT et al. Antidepressant-induced ubiquitination and degradation of the cardiac potassium channel hERG.
J. Biol. Chem., 2011 Sep 30 , 286 (34413-25).

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

Golding MC et al. Depletion of Kcnq1ot1 non-coding RNA does not affect imprinting maintenance in stem cells.
Development, 2011 Sep , 138 (3667-78).

588

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).

589

Digby GC et al. Acquired long QT interval: a case series of multifactorial QT prolongation.
Clin Cardiol, 2011 Sep , 34 (577-82).

590

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).

591

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).

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

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).

600

Tester DJ et al. Unexplained drownings and the cardiac channelopathies: a molecular autopsy series.
Mayo Clin. Proc., 2011 Oct , 86 (941-7).

601

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).

602

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).

603

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).

604

Kanda VA et al. Protein kinase C downregulates I(Ks) by stimulating KCNQ1-KCNE1 potassium channel endocytosis.
Heart Rhythm, 2011 Oct , 8 (1641-7).

605

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).

606

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).

607

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).

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

Nakajo K et al. KCNQ1 subdomains involved in KCNE modulation revealed by an invertebrate KCNQ1 orthologue.
J. Gen. Physiol., 2011 Nov , 138 (521-35).

614

Parvez B et al. The "missing" link in atrial fibrillation heritability.
J Electrocardiol, 2011 Nov , 44 (641-4).