Channelpedia

PubMed 24931202


Referenced in Channelpedia wiki pages of: none

Automatically associated channels: KCNQ1 , Kv7.1 , Nav1.5



Title: Sodium channels, cardiac arrhythmia, and therapeutic strategy.

Authors: Dori Miller, Lili Wang, Juming Zhong

Journal, date & volume: Adv. Pharmacol., 2014 , 70, 367-92

PubMed link: http://www.ncbi.nlm.nih.gov/pubmed/24931202


Abstract
Cardiac sodium channels are transmembrane proteins distributed in atrial and ventricular myocytes and Purkinje fibers. A large and rapid Na(+) influx through these channels initiates action potential and thus excitation-contraction coupling of cardiac cells. Cardiac sodium channel is composed of a pore-forming α-subunit and one or two accessory β-subunits. The cardiac α-subunit is encoded by gene SCN5A located on chromosome 3p21. There are four types of β-subunits identified so far, and β1 is the primary β-subunit in cardiac Na(+) channels. The gene responsible for β1 subunits is SCNB. The expression of β-subunits together with α subunits enhances the Na(+) current and modifies the channel activities. In addition, interactions of the cardiac Na(+) channel with other proteins may facilitate the channel activity and membrane expression of the channel. Over the past two decades, molecular genetic studies have identified the linkage of gene mutations of the Na(+) channel proteins and other regulatory proteins to many inherited arrhythmogenic diseases. The most common cardiac arrhythmogenic diseases associated with Na(+) channelopathies are long QT syndrome (LQT3) and Brugada syndromes (BrSs). This chapter intends to summarize the current understanding of the normal sodium-channel structure and function, the gene mutation-associated cardiac arrhythmias, and the current diagnosis and management of these diseases.