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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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Detalles Bibliográficos
Autores principales: Pesce, Emanuela, Sondo, Elvira, Ferrera, Loretta, Tomati, Valeria, Caci, Emanuela, Scudieri, Paolo, Musante, Ilaria, Renda, Mario, Baatallah, Nesrine, Servel, Nathalie, Hinzpeter, Alexandre, di Bernardo, Diego, Pedemonte, Nicoletta, Galietta, Luis J. V.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2018
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6300570/
https://www.ncbi.nlm.nih.gov/pubmed/30618756
http://dx.doi.org/10.3389/fphar.2018.01464