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Channelopathies from mutations in the cardiac sodium channel protein complex.

ABSTRACT: 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".

SUBMITTER: Adsit GS

PROVIDER: S-EPMC3720718 | biostudies-literature | 2013 Aug

REPOSITORIES: biostudies-literature

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Channelopathies from mutations in the cardiac sodium channel protein complex.

Adsit Graham S GS Vaidyanathan Ravi R Galler Carla M CM Kyle John W JW Makielski Jonathan C JC

Journal of molecular and cellular cardiology 20130401

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 wi ...[more]

PMID: 23557754

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