Referenced in: none

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

Title: Genotype and severity of long QT syndrome.

Authors: J A Towbin, Z Wang, H Li

Journal, date & volume: Drug Metab. Dispos., 2001 Apr , 29, 574-9

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/11259355

Abstract
Sudden cardiac death occurs in the United States with an incidence greater than 300,000 persons per year. The underlying cause of death is commonly considered to be due to primary or secondary arrhythmias. In cases in which no structural heart disease can be identified, the long QT syndromes (LQTS) are now commonly considered as likely causes. Multiple genes causing LQTS have been identified thus far, all encoding cardiac ion channels. These include two potassium channel alpha-subunits (KVLQT1, HERG), two potassium channel beta-subunits (minK, MiRP1), and one sodium channel gene (SCN5A). The purpose of this review is to describe the current understanding of the molecular genetics of LQTS and the resultant phenotypes.