Molecular Pathophysiology of Congenital Long QT Syndrome.

ABSTRACT: Ion channels represent the molecular entities that give rise to the cardiac action potential, the fundamental cellular electrical event in the heart. The concerted function of these channels leads to normal cyclical excitation and resultant contraction of cardiac muscle. Research into cardiac ion channel regulation and mutations that underlie disease pathogenesis has greatly enhanced our knowledge of the causes and clinical management of cardiac arrhythmia. Here we review the molecular determinants, pathogenesis, and pharmacology of congenital Long QT Syndrome. We examine mechanisms of dysfunction associated with three critical cardiac currents that comprise the majority of congenital Long QT Syndrome cases: 1) I_Ks, the slow delayed rectifier current; 2) I_Kr, the rapi

SUBMITTER: Bohnen MS 

PROVIDER: S-EPMC5539372 | biostudies-literature | 2017 Jan

REPOSITORIES: biostudies-literature