» Articles » PMID: 23557754

Channelopathies from Mutations in the Cardiac Sodium Channel Protein Complex

Overview
Date 2013 Apr 6
PMID 23557754
Citations 25
Authors
Affiliations
Soon will be listed here.
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".

Citing Articles

Protein 14-3-3 Influences the Response of the Cardiac Sodium Channel Na1.5 to Antiarrhythmic Drugs.

Zheng Y, Deschenes I J Pharmacol Exp Ther. 2022; 384(3):417-428.

PMID: 36460339 PMC: 9976794. DOI: 10.1124/jpet.122.001407.


Differential Effect of Three Macrolide Antibiotics on Cardiac Pathology and Electrophysiology in a Myocardial Infarction Rat Model: Influence on Sodium Nav1.5 Channel Expression.

Farag N, El-Kherbetawy M, Ismail H, Abdelrady A, Toraih E, Abdelbasset W Pharmaceuticals (Basel). 2021; 14(7).

PMID: 34206182 PMC: 8308720. DOI: 10.3390/ph14070597.


Long QT syndrome - Bench to bedside.

Ponce-Balbuena D, Deschenes I Heart Rhythm O2. 2021; 2(1):89-106.

PMID: 34113909 PMC: 8183884. DOI: 10.1016/j.hroo.2021.01.006.


Na1.2 EFL domain allosterically enhances Ca binding to sites I and II of WT and pathogenic calmodulin mutants bound to the channel CTD.

Mahling R, Hovey L, Isbell H, Marx D, Miller M, Kilpatrick A Structure. 2021; 29(12):1339-1356.e7.

PMID: 33770503 PMC: 8458505. DOI: 10.1016/j.str.2021.03.002.


Cardiac small-conductance calcium-activated potassium channels in health and disease.

Zhang X, Thai P, Lieu D, Chiamvimonvat N Pflugers Arch. 2021; 473(3):477-489.

PMID: 33624131 PMC: 7940285. DOI: 10.1007/s00424-021-02535-0.


References
1.
Milstein M, Musa H, Balbuena D, Anumonwo J, Auerbach D, Furspan P . Dynamic reciprocity of sodium and potassium channel expression in a macromolecular complex controls cardiac excitability and arrhythmia. Proc Natl Acad Sci U S A. 2012; 109(31):E2134-43. PMC: 3412015. DOI: 10.1073/pnas.1109370109. View

2.
Ou Y, Strege P, Miller S, Makielski J, Ackerman M, Gibbons S . Syntrophin gamma 2 regulates SCN5A gating by a PDZ domain-mediated interaction. J Biol Chem. 2002; 278(3):1915-23. DOI: 10.1074/jbc.M209938200. View

3.
Murray K, Hu N, Daw J, Shin H, Watson M, Mashburn A . Functional effects of protein kinase C activation on the human cardiac Na+ channel. Circ Res. 1997; 80(3):370-6. DOI: 10.1161/01.res.80.3.370. View

4.
Spampanato J, Kearney J, de Haan G, McEwen D, Escayg A, Aradi I . A novel epilepsy mutation in the sodium channel SCN1A identifies a cytoplasmic domain for beta subunit interaction. J Neurosci. 2004; 24(44):10022-34. PMC: 6730248. DOI: 10.1523/JNEUROSCI.2034-04.2004. View

5.
Goldin A, Barchi R, Caldwell J, Hofmann F, Howe J, Hunter J . Nomenclature of voltage-gated sodium channels. Neuron. 2001; 28(2):365-8. DOI: 10.1016/s0896-6273(00)00116-1. View