CFTR Protein: Not Just a Chloride Channel?

Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR protein is known to acts as a chloride (Cl<sup>−</sup>) channel expressed in the exocrine glands of several bod...

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Autores principales:	Laurence S. Hanssens, Jean Duchateau, Georges J. Casimir
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	cystic fibrosis CFTR protein channel chloride bicarbonate glutathione Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/d265e9e8330342e58ed891de8c5be39f
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spelling	oai:doaj.org-article:d265e9e8330342e58ed891de8c5be39f2021-11-25T17:07:51ZCFTR Protein: Not Just a Chloride Channel?10.3390/cells101128442073-4409https://doaj.org/article/d265e9e8330342e58ed891de8c5be39f2021-10-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/2844https://doaj.org/toc/2073-4409Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR protein is known to acts as a chloride (Cl<sup>−</sup>) channel expressed in the exocrine glands of several body systems where it also regulates other ion channels, including the epithelial sodium (Na<sup>+</sup>) channel (ENaC) that plays a key role in salt absorption. This function is crucial to the osmotic balance of the mucus and its viscosity. However, the pathophysiology of CF is more challenging than a mere dysregulation of epithelial ion transport, mainly resulting in impaired mucociliary clearance (MCC) with consecutive bronchiectasis and in exocrine pancreatic insufficiency. This review shows that the CFTR protein is not just a chloride channel. For a long time, research in CF has focused on abnormal Cl<sup>−</sup> and Na<sup>+</sup> transport. Yet, the CFTR protein also regulates numerous other pathways, such as the transport of HCO<sub>3</sub><sup>−</sup>, glutathione and thiocyanate, immune cells, and the metabolism of lipids. It influences the pH homeostasis of airway surface liquid and thus the MCC as well as innate immunity leading to chronic infection and inflammation, all of which are considered as key pathophysiological characteristics of CF.Laurence S. HanssensJean DuchateauGeorges J. CasimirMDPI AGarticlecystic fibrosisCFTR proteinchannelchloridebicarbonateglutathioneBiology (General)QH301-705.5ENCells, Vol 10, Iss 2844, p 2844 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	cystic fibrosis CFTR protein channel chloride bicarbonate glutathione Biology (General) QH301-705.5
spellingShingle	cystic fibrosis CFTR protein channel chloride bicarbonate glutathione Biology (General) QH301-705.5 Laurence S. Hanssens Jean Duchateau Georges J. Casimir CFTR Protein: Not Just a Chloride Channel?
description	Cystic fibrosis (CF) is a recessive genetic disease caused by mutations in a gene encoding a protein called Cystic Fibrosis Transmembrane Conductance Regulator (CFTR). The CFTR protein is known to acts as a chloride (Cl<sup>−</sup>) channel expressed in the exocrine glands of several body systems where it also regulates other ion channels, including the epithelial sodium (Na<sup>+</sup>) channel (ENaC) that plays a key role in salt absorption. This function is crucial to the osmotic balance of the mucus and its viscosity. However, the pathophysiology of CF is more challenging than a mere dysregulation of epithelial ion transport, mainly resulting in impaired mucociliary clearance (MCC) with consecutive bronchiectasis and in exocrine pancreatic insufficiency. This review shows that the CFTR protein is not just a chloride channel. For a long time, research in CF has focused on abnormal Cl<sup>−</sup> and Na<sup>+</sup> transport. Yet, the CFTR protein also regulates numerous other pathways, such as the transport of HCO<sub>3</sub><sup>−</sup>, glutathione and thiocyanate, immune cells, and the metabolism of lipids. It influences the pH homeostasis of airway surface liquid and thus the MCC as well as innate immunity leading to chronic infection and inflammation, all of which are considered as key pathophysiological characteristics of CF.
format	article
author	Laurence S. Hanssens Jean Duchateau Georges J. Casimir
author_facet	Laurence S. Hanssens Jean Duchateau Georges J. Casimir
author_sort	Laurence S. Hanssens
title	CFTR Protein: Not Just a Chloride Channel?
title_short	CFTR Protein: Not Just a Chloride Channel?
title_full	CFTR Protein: Not Just a Chloride Channel?
title_fullStr	CFTR Protein: Not Just a Chloride Channel?
title_full_unstemmed	CFTR Protein: Not Just a Chloride Channel?
title_sort	cftr protein: not just a chloride channel?
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/d265e9e8330342e58ed891de8c5be39f
work_keys_str_mv	AT laurenceshanssens cftrproteinnotjustachloridechannel AT jeanduchateau cftrproteinnotjustachloridechannel AT georgesjcasimir cftrproteinnotjustachloridechannel
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CFTR Protein: Not Just a Chloride Channel?

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