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Structural mechanisms of CFTR function and dysfunction

Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) chloride channel plays a critical role in regulating transepithelial movement of water and electrolyte in exocrine tissues. Malfunction of the channel because of mutations of the cftr gene results in CF, the most prevalent lethal geneti...

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Autores principales: Hwang, Tzyh-Chang, Yeh, Jiunn-Tyng, Zhang, Jingyao, Yu, Ying-Chun, Yeh, Han-I, Destefano, Samantha
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Rockefeller University Press 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5881446/
https://www.ncbi.nlm.nih.gov/pubmed/29581173
http://dx.doi.org/10.1085/jgp.201711946
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author Hwang, Tzyh-Chang
Yeh, Jiunn-Tyng
Zhang, Jingyao
Yu, Ying-Chun
Yeh, Han-I
Destefano, Samantha
author_facet Hwang, Tzyh-Chang
Yeh, Jiunn-Tyng
Zhang, Jingyao
Yu, Ying-Chun
Yeh, Han-I
Destefano, Samantha
author_sort Hwang, Tzyh-Chang
collection PubMed
description Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) chloride channel plays a critical role in regulating transepithelial movement of water and electrolyte in exocrine tissues. Malfunction of the channel because of mutations of the cftr gene results in CF, the most prevalent lethal genetic disease among Caucasians. Recently, the publication of atomic structures of CFTR in two distinct conformations provides, for the first time, a clear overview of the protein. However, given the highly dynamic nature of the interactions among CFTR’s various domains, better understanding of the functional significance of these structures requires an integration of these new structural insights with previously established biochemical/biophysical studies, which is the goal of this review.
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spelling pubmed-58814462018-10-02 Structural mechanisms of CFTR function and dysfunction Hwang, Tzyh-Chang Yeh, Jiunn-Tyng Zhang, Jingyao Yu, Ying-Chun Yeh, Han-I Destefano, Samantha J Gen Physiol Reviews Cystic fibrosis (CF) transmembrane conductance regulator (CFTR) chloride channel plays a critical role in regulating transepithelial movement of water and electrolyte in exocrine tissues. Malfunction of the channel because of mutations of the cftr gene results in CF, the most prevalent lethal genetic disease among Caucasians. Recently, the publication of atomic structures of CFTR in two distinct conformations provides, for the first time, a clear overview of the protein. However, given the highly dynamic nature of the interactions among CFTR’s various domains, better understanding of the functional significance of these structures requires an integration of these new structural insights with previously established biochemical/biophysical studies, which is the goal of this review. Rockefeller University Press 2018-04-02 /pmc/articles/PMC5881446/ /pubmed/29581173 http://dx.doi.org/10.1085/jgp.201711946 Text en © 2018 Hwang et al. http://www.rupress.org/terms/https://creativecommons.org/licenses/by-nc-sa/4.0/This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.rupress.org/terms/). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 4.0 International license, as described at https://creativecommons.org/licenses/by-nc-sa/4.0/).
spellingShingle Reviews
Hwang, Tzyh-Chang
Yeh, Jiunn-Tyng
Zhang, Jingyao
Yu, Ying-Chun
Yeh, Han-I
Destefano, Samantha
Structural mechanisms of CFTR function and dysfunction
title Structural mechanisms of CFTR function and dysfunction
title_full Structural mechanisms of CFTR function and dysfunction
title_fullStr Structural mechanisms of CFTR function and dysfunction
title_full_unstemmed Structural mechanisms of CFTR function and dysfunction
title_short Structural mechanisms of CFTR function and dysfunction
title_sort structural mechanisms of cftr function and dysfunction
topic Reviews
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5881446/
https://www.ncbi.nlm.nih.gov/pubmed/29581173
http://dx.doi.org/10.1085/jgp.201711946
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