The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis

In cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response...

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Autores principales:	Pascal Trouvé, Claude Férec, Emmanuelle Génin
Formato:	article
Lenguaje:	EN
Publicado:	MDPI AG 2021
Materias:	cystic fibrosis unfolded protein response inflammation infection Biology (General) QH301-705.5
Acceso en línea:	https://doaj.org/article/30f8e4ee39904309b41976d16eda6351
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id	oai:doaj.org-article:30f8e4ee39904309b41976d16eda6351
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spelling	oai:doaj.org-article:30f8e4ee39904309b41976d16eda63512021-11-25T17:10:00ZThe Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis10.3390/cells101129802073-4409https://doaj.org/article/30f8e4ee39904309b41976d16eda63512021-11-01T00:00:00Zhttps://www.mdpi.com/2073-4409/10/11/2980https://doaj.org/toc/2073-4409In cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response (UPR) involving ATF6, which reduces the expression of p.Phe508del-CFTR. There are still some debates on the role of the UPR in CF: could it be triggered by the accumulation of misfolded CFTR proteins in the endoplasmic reticulum as was proposed for the most common CFTR mutation p.Phe508del? Or, is it the consequence of inflammation and infection that occur in the disease? In this review, we summarize recent findings on UPR in CF and show how infection, inflammation and UPR act together in CF. We propose to rethink their respective role in CF and to consider them as a whole.Pascal TrouvéClaude FérecEmmanuelle GéninMDPI AGarticlecystic fibrosisunfolded protein responseinflammationinfectionBiology (General)QH301-705.5ENCells, Vol 10, Iss 2980, p 2980 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	cystic fibrosis unfolded protein response inflammation infection Biology (General) QH301-705.5
spellingShingle	cystic fibrosis unfolded protein response inflammation infection Biology (General) QH301-705.5 Pascal Trouvé Claude Férec Emmanuelle Génin The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
description	In cystic fibrosis (CF), p.Phe508del is the most frequent mutation in the Cystic Fibrosis Transmembrane conductance Regulator (<i>CFTR</i>) gene. The p.Phe508del-CFTR protein is retained in the ER and rapidly degraded. This retention likely triggers an atypical Unfolded Protein Response (UPR) involving ATF6, which reduces the expression of p.Phe508del-CFTR. There are still some debates on the role of the UPR in CF: could it be triggered by the accumulation of misfolded CFTR proteins in the endoplasmic reticulum as was proposed for the most common CFTR mutation p.Phe508del? Or, is it the consequence of inflammation and infection that occur in the disease? In this review, we summarize recent findings on UPR in CF and show how infection, inflammation and UPR act together in CF. We propose to rethink their respective role in CF and to consider them as a whole.
format	article
author	Pascal Trouvé Claude Férec Emmanuelle Génin
author_facet	Pascal Trouvé Claude Férec Emmanuelle Génin
author_sort	Pascal Trouvé
title	The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
title_short	The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
title_full	The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
title_fullStr	The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
title_full_unstemmed	The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis
title_sort	interplay between the unfolded protein response, inflammation and infection in cystic fibrosis
publisher	MDPI AG
publishDate	2021
url	https://doaj.org/article/30f8e4ee39904309b41976d16eda6351
work_keys_str_mv	AT pascaltrouve theinterplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis AT claudeferec theinterplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis AT emmanuellegenin theinterplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis AT pascaltrouve interplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis AT claudeferec interplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis AT emmanuellegenin interplaybetweentheunfoldedproteinresponseinflammationandinfectionincysticfibrosis
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The Interplay between the Unfolded Protein Response, Inflammation and Infection in Cystic Fibrosis

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