Referenced in: none

Automatically associated channels: Kv11.1 , Kv7.1 , Nav1.5

Title: The molecular basis of long QT syndrome and prospects for therapy.

Authors: Q Wang, N E Bowles, J A Towbin

Journal, date & volume: , 1998 Sep , 4, 382-8

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/9791861

Abstract
Long QT syndrome (LQT) is a cardiac disorder that causes sudden death from ventricular tachyarrhythmias, specifically torsade de pointes. Two types of LQT have been reported, autosomal-dominant LQT (Romano-Ward syndrome) and autosomal-recessive LQT (Jervell and Lange-Nielsen syndrome); Jervell and Lange-Nielsen syndrome is also associated with deafness. Four LQT genes have been identified for autosomal-dominant LQT: K+ channel genes KVLQT1 on chromosome 11p15.5, HERG on 7q35-36 and minK on 21q22, and the cardiac Na+ channel gene SCN5A on chromosome 3p21-24. Two genes, KVLQT1 and minK, have been identified for Jervell and Lange-Nielsen syndrome. Genetic testing and gene-specific therapies are available for some LQT patients.