Molecular mechanisms underlying the long QT syndrome.
Recent studies of the molecular basis of the long QT syndrome (LQTS) have advanced our understanding of the mechanisms responsible for the abnormal prolongation of ventricular repolarization and revealed associations between LQTS and other primary electrical diseases of the heart such as Brugada syndrome. The role of DNA single nucleotide polymorphisms in acquired LQTS and differences between the Romano-Ward and Jervell-Lange-Nielsen forms of congenital LQTS are gradually coming into focus. In this brief review, our goal is to summarize the molecular mechanisms proposed to underlie the susceptibility to arrhythmias in LQTS and discuss the direction of current and future research.
Dumaine
Robert
R
Masonic Medical Research Laboratory, Utica, New York 13501, USA. rdumaine@mmrl.edu
Antzelevitch
Charles
C
D005192:Family Health; D006801:Humans; D007473:Ion Channels / Q000235:genetics; D008133:Long QT Syndrome / Q000235:genetics*; D008967:Molecular Biology; D009154:Mutation / Q000235:genetics
D007473:Ion Channels