Department of Medicine, Division of Cardiovascular Medicine, University of Wisconsin, Madison, WI 53792, USA.
J Mol Cell Cardiol. 2013 Aug;61:34-43. doi: 10.1016/j.yjmcc.2013.03.017. Epub 2013 Apr 1.
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".
心脏的钠离子电流是心脏兴奋性的基础，调节和传导这种电流的蛋白质的遗传异常会导致遗传性心律失常综合征。本综述重点介绍了导致心律失常的钠通道复合物中非孔形成蛋白的遗传突变，以及它们影响心脏钠电流功能和障碍的推断机制。定义这些复合物的结构和功能以及它们的调节方式将有助于了解该复合物在正常和异常生理及动态平衡中的可能作用。本文是题为“心肌细胞中的 Na(+) 调节”特刊的一部分。
