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

• Main Authors: Emanuela Pesce (Author), Elvira Sondo (Author), Loretta Ferrera (Author), Valeria Tomati (Author), Emanuela Caci (Author), Paolo Scudieri (Author), Ilaria Musante (Author), Mario Renda (Author), Nesrine Baatallah (Author), Nathalie Servel (Author), Alexandre Hinzpeter (Author), Diego di Bernardo (Author), Nicoletta Pedemonte (Author), Luis J. V. Galietta (Author)
• Format: Knjiga
• Izdano: Frontiers Media S.A., 2018-12-01T00:00:00Z.
• Teme: article
• Online dostop: Connect to this object online.

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...