Channelopathies from mutations in the cardiac sodium channel protein complex

被引:37
作者
Adsit, Graham S. [1 ]
Vaidyanathan, Ravi [1 ]
Galler, Carla M. [2 ]
Kyle, John W. [1 ]
Makielski, Jonathan C. [1 ]
机构
[1] Univ Wisconsin, Dept Med, Div Cardiovasc Med, Madison, WI 53792 USA
[2] Madison Coll, Div Visual Commun, Sch Business & Appl Arts, Madison, WI 53704 USA
关键词
Sodium current; SCN5A; Arrhythmia; Long QT syndrome; Brugada Syndrome; SIDS; VOLTAGE-GATED SODIUM; NITRIC-OXIDE SYNTHASE; SARCOLEMMAL CALCIUM-PUMP; BETA-SUBUNIT SCN3B; LONG QT SYNDROME; NA+ CHANNEL; BRUGADA-SYNDROME; ALPHA-SUBUNITS; ION-CHANNEL; ANKYRIN-G;
D O I
10.1016/j.yjmcc.2013.03.017
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
The cardiac sodium current underlies excitability in heart, and inherited abnormalities of the proteins regulating and conducting this current cause inherited arrhythmia syndromes. This review focuses on inherited mutations in non-pore forming proteins of sodium channel complexes that cause cardiac arrhythmia, and the deduced mechanisms by which they affect function and dysfunction of the cardiac sodium current. Defining the structure and function of these complexes and how they are regulated will contribute to understanding the possible roles for this complex in normal and abnormal physiology and homeostasis. This article is part of a Special Issue entitled "Na+ Regulation in Cardiac Myocytes". (C) 2013 Elsevier Ltd. All rights reserved.
引用
收藏
页码:34 / 43
页数:10
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