The Distribution and Role of the CFTR Protein in the Intracellular Compartments

Cystic fibrosis is a hereditary disease that mainly affects secretory organs in humans. It is caused by mutations in the gene encoding CFTR with the most common phenylalanine deletion at position 508. CFTR is an anion channel mainly conducting Cl<sup>−</sup> across the apical membranes o...

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Autores principales:	Agnieszka Lukasiak, Miroslaw Zajac
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	chloride channels cystic fibrosis transmembrane conductance regulator ion transport intracellular organelle Chemical technology TP1-1185 Chemical engineering TP155-156
Acceso en línea:	https://doaj.org/article/81cd0c7859ed4952a31a09623e9369ea
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spelling	oai:doaj.org-article:81cd0c7859ed4952a31a09623e9369ea2021-11-25T18:19:28ZThe Distribution and Role of the CFTR Protein in the Intracellular Compartments10.3390/membranes111108042077-0375https://doaj.org/article/81cd0c7859ed4952a31a09623e9369ea2021-10-01T00:00:00Zhttps://www.mdpi.com/2077-0375/11/11/804https://doaj.org/toc/2077-0375Cystic fibrosis is a hereditary disease that mainly affects secretory organs in humans. It is caused by mutations in the gene encoding CFTR with the most common phenylalanine deletion at position 508. CFTR is an anion channel mainly conducting Cl<sup>−</sup> across the apical membranes of many different epithelial cells, the impairment of which causes dysregulation of epithelial fluid secretion and thickening of the mucus. This, in turn, leads to the dysfunction of organs such as the lungs, pancreas, kidney and liver. The CFTR protein is mainly localized in the plasma membrane; however, there is a growing body of evidence that it is also present in the intracellular organelles such as the endosomes, lysosomes, phagosomes and mitochondria. Dysfunction of the CFTR protein affects not only the ion transport across the epithelial tissues, but also has an impact on the proper functioning of the intracellular compartments. The review aims to provide a summary of the present state of knowledge regarding CFTR localization and function in intracellular compartments, the physiological role of this localization and the consequences of protein dysfunction at cellular, epithelial and organ levels. An in-depth understanding of intracellular processes involved in CFTR impairment may reveal novel opportunities in pharmacological agents of cystic fibrosis.Agnieszka LukasiakMiroslaw ZajacMDPI AGarticlechloride channelscystic fibrosis transmembrane conductance regulatorion transportintracellular organelleChemical technologyTP1-1185Chemical engineeringTP155-156ENMembranes, Vol 11, Iss 804, p 804 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	chloride channels cystic fibrosis transmembrane conductance regulator ion transport intracellular organelle Chemical technology TP1-1185 Chemical engineering TP155-156
spellingShingle	chloride channels cystic fibrosis transmembrane conductance regulator ion transport intracellular organelle Chemical technology TP1-1185 Chemical engineering TP155-156 Agnieszka Lukasiak Miroslaw Zajac The Distribution and Role of the CFTR Protein in the Intracellular Compartments
description	Cystic fibrosis is a hereditary disease that mainly affects secretory organs in humans. It is caused by mutations in the gene encoding CFTR with the most common phenylalanine deletion at position 508. CFTR is an anion channel mainly conducting Cl<sup>−</sup> across the apical membranes of many different epithelial cells, the impairment of which causes dysregulation of epithelial fluid secretion and thickening of the mucus. This, in turn, leads to the dysfunction of organs such as the lungs, pancreas, kidney and liver. The CFTR protein is mainly localized in the plasma membrane; however, there is a growing body of evidence that it is also present in the intracellular organelles such as the endosomes, lysosomes, phagosomes and mitochondria. Dysfunction of the CFTR protein affects not only the ion transport across the epithelial tissues, but also has an impact on the proper functioning of the intracellular compartments. The review aims to provide a summary of the present state of knowledge regarding CFTR localization and function in intracellular compartments, the physiological role of this localization and the consequences of protein dysfunction at cellular, epithelial and organ levels. An in-depth understanding of intracellular processes involved in CFTR impairment may reveal novel opportunities in pharmacological agents of cystic fibrosis.
format	article
author	Agnieszka Lukasiak Miroslaw Zajac
author_facet	Agnieszka Lukasiak Miroslaw Zajac
author_sort	Agnieszka Lukasiak
title	The Distribution and Role of the CFTR Protein in the Intracellular Compartments
title_short	The Distribution and Role of the CFTR Protein in the Intracellular Compartments
title_full	The Distribution and Role of the CFTR Protein in the Intracellular Compartments
title_fullStr	The Distribution and Role of the CFTR Protein in the Intracellular Compartments
title_full_unstemmed	The Distribution and Role of the CFTR Protein in the Intracellular Compartments
title_sort	distribution and role of the cftr protein in the intracellular compartments
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/81cd0c7859ed4952a31a09623e9369ea
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The Distribution and Role of the CFTR Protein in the Intracellular Compartments

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