The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism

The Autophagy Inhibitor Spautin-1 Antagonizes Rescue of Mutant CFTR Through an Autophagy-Independent and USP13-Mediated Mechanism

The mutation F508del, responsible for a majority of cystic fibrosis cases, provokes the instability and misfolding of the CFTR chloride channel. Pharmacological recovery of F508del-CFTR may be obtained with small molecules called correctors. However, treatment with a single corrector in vivo and in...

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Bibliographic Details
Main Authors: Emanuela Pesce, Elvira Sondo, Loretta Ferrera, Valeria Tomati, Emanuela Caci, Paolo Scudieri, Ilaria Musante, Mario Renda, Nesrine Baatallah, Nathalie Servel, Alexandre Hinzpeter, Diego di Bernardo, Nicoletta Pedemonte, Luis J. V. Galietta
Format: Article
Language:English
Published: Frontiers Media S.A. 2018-12-01
Series:Frontiers in Pharmacology
Subjects:
CFTR
chloride channel
cystic fibrosis
autophagy
ubiquitination
spautin-1
Online Access:https://www.frontiersin.org/article/10.3389/fphar.2018.01464/full

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https://www.frontiersin.org/article/10.3389/fphar.2018.01464/full

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