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".
Keywords:
Arrhythmia; Brugada Syndrome; Long QT syndrome; SCN5A; SIDS; Sodium current.
Copyright © 2013 Elsevier Ltd. All rights reserved.
Publication types
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Research Support, N.I.H., Extramural
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Review
MeSH terms
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Animals
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Arrhythmias, Cardiac / genetics
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Arrhythmias, Cardiac / metabolism
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Calcium-Binding Proteins / genetics
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Calcium-Binding Proteins / metabolism
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Channelopathies / genetics*
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Channelopathies / metabolism
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Channelopathies / physiopathology
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Homeostasis
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Humans
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Membrane Proteins / genetics
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Membrane Proteins / metabolism
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Multiprotein Complexes / genetics
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Multiprotein Complexes / metabolism
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Muscle Proteins / genetics
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Muscle Proteins / metabolism
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Mutation, Missense*
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Myocardial Contraction
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NAV1.5 Voltage-Gated Sodium Channel / genetics*
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NAV1.5 Voltage-Gated Sodium Channel / metabolism
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Protein Subunits / genetics
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Protein Subunits / metabolism
Substances
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Calcium-Binding Proteins
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Membrane Proteins
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Multiprotein Complexes
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Muscle Proteins
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NAV1.5 Voltage-Gated Sodium Channel
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Protein Subunits
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SCN5A protein, human
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syntrophin alpha1