Referenced in: none

Automatically associated channels: Kv7.1

Title: Human congenital long QT syndrome: more than previously thought?

Authors: Bernard Attali

Journal, date & volume: Trends Pharmacol. Sci., 2002 Jun , 23, 249-51

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/12084623

Abstract
Mutations in KCNQ1 and KCNE1, the alpha- and beta-subunits of the I(KS) K+ channel, produce the cardiac long QT (LQT) syndrome. These subunits are expressed in heart and inner ear, but also in epithelial tissues such as kidney or intestine where their functional roles have remained elusive. Recent work has shown that KCNE1-deficient mice display chronic hypokalemia and hyperaldosteronism. These results have significant implications for human congenital LQT syndromes because hypokalemia increases the risk of ventricular arrhythmia and cardiac sudden death.