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Inherited Cardiac Arrhythmia Syndromes: Focus on Molecular Mechanisms Underlying TRPM4 Channelopathies.
- Source :
-
Cardiovascular therapeutics [Cardiovasc Ther] 2020 Dec 16; Vol. 2020, pp. 6615038. Date of Electronic Publication: 2020 Dec 16 (Print Publication: 2020). - Publication Year :
- 2020
-
Abstract
- The Transient Receptor Potential Melastatin 4 (TRPM4) is a transmembrane N-glycosylated ion channel that belongs to the large family of TRP proteins. It has an equal permeability to Na <superscript>+</superscript> and K <superscript>+</superscript> and is activated via an increase of the intracellular calcium concentration and membrane depolarization. Due to its wide distribution, TRPM4 dysfunction has been linked with several pathophysiological processes, including inherited cardiac arrhythmias. Many pathogenic variants of the TRPM4 gene have been identified in patients with different forms of cardiac disorders such as conduction defects, Brugada syndrome, and congenital long QT syndrome. At the cellular level, these variants induce either gain- or loss-of-function of TRPM4 channels for similar clinical phenotypes. However, the molecular mechanisms associating these functional alterations to the clinical phenotypes remain poorly understood. The main objective of this article is to review the major cardiac TRPM4 channelopathies and recent advances regarding their genetic background and the underlying molecular mechanisms.<br />Competing Interests: Prof. Mohamed Yassine Amarouch declares that he has no conflict of interest. Prof. Jaouad El Hilaly declares that he has no conflict of interest.<br /> (Copyright © 2020 Mohamed-Yassine Amarouch and Jaouad El Hilaly.)
- Subjects :
- Animals
Arrhythmias, Cardiac etiology
Arrhythmias, Cardiac physiopathology
Brugada Syndrome genetics
Brugada Syndrome physiopathology
Channelopathies complications
Channelopathies physiopathology
Genetic Predisposition to Disease
Heredity
Humans
Long QT Syndrome genetics
Long QT Syndrome physiopathology
Pedigree
Phenotype
Arrhythmias, Cardiac genetics
Channelopathies genetics
Heart Rate genetics
TRPM Cation Channels genetics
Subjects
Details
- Language :
- English
- ISSN :
- 1755-5922
- Volume :
- 2020
- Database :
- MEDLINE
- Journal :
- Cardiovascular therapeutics
- Publication Type :
- Academic Journal
- Accession number :
- 33381229
- Full Text :
- https://doi.org/10.1155/2020/6615038