Structures Illuminate Cardiac Ion Channel Functions in Health and in Long QT Syndrome.

ABSTRACT: The cardiac action potential is critical to the production of a synchronized heartbeat. This electrical impulse is governed by the intricate activity of cardiac ion channels, among them the cardiac voltage-gated potassium (K_v) channels KCNQ1 and hERG as well as the voltage-gated sodium (Na_v) channel encoded by SCN5A. Each channel performs a highly distinct function, despite sharing a common topology and structural components. These three channels are also the primary proteins mutated in congenital long QT syndrome (LQTS), a genetic condition that predisposes to cardiac arrhythmia and sudden cardiac death due to impaired repolarization of the action potential and has a particular proclivity for reentrant ventricular arrhythmias. Recent cryo-electron microscopy s

SUBMITTER: Brewer KR 

PROVIDER: S-EPMC7212895 | biostudies-literature | 2020

REPOSITORIES: biostudies-literature