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Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy
Voltage-gated ion channels (VGIC) are transmembrane proteins responsible for the generation of electrical signals in excitable cells. VGIC were first described in 1952 by Hodgkin and Huxley,(1) and have since been associated with various physiological functions such as propagating nerve impulses, lo...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Taylor & Francis
2015
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Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4594536/ https://www.ncbi.nlm.nih.gov/pubmed/26046592 http://dx.doi.org/10.1080/19336950.2015.1031937 |
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author | Moreau, Adrien Gosselin-Badaroudine, Pascal Chahine, Mohamed |
author_facet | Moreau, Adrien Gosselin-Badaroudine, Pascal Chahine, Mohamed |
author_sort | Moreau, Adrien |
collection | PubMed |
description | Voltage-gated ion channels (VGIC) are transmembrane proteins responsible for the generation of electrical signals in excitable cells. VGIC were first described in 1952 by Hodgkin and Huxley,(1) and have since been associated with various physiological functions such as propagating nerve impulses, locomotion, and cardiac excitability. VGIC include channels specialized in the selective passage of K(+), Ca(2+) Na(+), or H(+). They are composed of 2 main structures: the pore domain (PD) and the voltage sensor domain (VSD). The PD ensures the physiological flow of ions and is typically composed of 8 transmembrane segments (TM). The VSD detects voltage variations and is composed of 4 TM (S1-S4). Given their crucial physiological role, VGIC dysfunctions are associated with diverse pathologies known as ion channelopathies. These dysfunctions usually affect the membrane expression of ion channels or voltage-dependent conformational changes of the pore. However, an increasing number of ion channelopathies, including periodic paralysis, dilated cardiomyopathy (DCM) associated with cardiac arrhythmias, and peripheral nerve hyperexcitability (PNH), have been linked to the appearance of a new pathological mechanism involving the creation of an alternative permeation pathway through the normally non-conductive VSD of VGIC. This permeation pathway is called the gating pore or omega pore. |
format | Online Article Text |
id | pubmed-4594536 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2015 |
publisher | Taylor & Francis |
record_format | MEDLINE/PubMed |
spelling | pubmed-45945362016-02-03 Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy Moreau, Adrien Gosselin-Badaroudine, Pascal Chahine, Mohamed Channels (Austin) Article Addendum Voltage-gated ion channels (VGIC) are transmembrane proteins responsible for the generation of electrical signals in excitable cells. VGIC were first described in 1952 by Hodgkin and Huxley,(1) and have since been associated with various physiological functions such as propagating nerve impulses, locomotion, and cardiac excitability. VGIC include channels specialized in the selective passage of K(+), Ca(2+) Na(+), or H(+). They are composed of 2 main structures: the pore domain (PD) and the voltage sensor domain (VSD). The PD ensures the physiological flow of ions and is typically composed of 8 transmembrane segments (TM). The VSD detects voltage variations and is composed of 4 TM (S1-S4). Given their crucial physiological role, VGIC dysfunctions are associated with diverse pathologies known as ion channelopathies. These dysfunctions usually affect the membrane expression of ion channels or voltage-dependent conformational changes of the pore. However, an increasing number of ion channelopathies, including periodic paralysis, dilated cardiomyopathy (DCM) associated with cardiac arrhythmias, and peripheral nerve hyperexcitability (PNH), have been linked to the appearance of a new pathological mechanism involving the creation of an alternative permeation pathway through the normally non-conductive VSD of VGIC. This permeation pathway is called the gating pore or omega pore. Taylor & Francis 2015-06-05 /pmc/articles/PMC4594536/ /pubmed/26046592 http://dx.doi.org/10.1080/19336950.2015.1031937 Text en © 2015 The Author(s). Published with license by Taylor & Francis Group, LLC http://creativecommons.org/licenses/by-nc/3.0/ This is an Open Access article distributed under the terms of the Creative Commons Attribution-Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. The moral rights of the named author(s) have been asserted. |
spellingShingle | Article Addendum Moreau, Adrien Gosselin-Badaroudine, Pascal Chahine, Mohamed Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title_full | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title_fullStr | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title_full_unstemmed | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title_short | Gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
title_sort | gating pore currents, a new pathological mechanism underlying cardiac arrhythmias associated with dilated cardiomyopathy |
topic | Article Addendum |
url | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4594536/ https://www.ncbi.nlm.nih.gov/pubmed/26046592 http://dx.doi.org/10.1080/19336950.2015.1031937 |
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