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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...
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. |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Frontiers Media S.A.
2018
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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 |
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