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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Bibliographic Details
Main Authors: Pascal Trouvé, Claude Férec, Emmanuelle Génin
Format: Article
Language:English
Published: MDPI AG 2021-11-01
Series:Cells
Subjects:
Online Access:https://www.mdpi.com/2073-4409/10/11/2980