Restoration of CFTR function in patients with cystic fibrosis carrying the F508del-CFTR mutation

Restoration of BECN1/Beclin 1-dependent autophagy and depletion of SQSTM1/p62 by genetic manipulation or autophagy-stimulatory proteostasis regulators, such as cystamine, have positive effects on mouse models of human cystic fibrosis (CF). These measures rescue the functional expression of the most...

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Detalles Bibliográficos
Autores principales: Stefano, Daniela De, Villella, Valeria R, Esposito, Speranza, Tosco, Antonella, Sepe, Angela, Gregorio, Fabiola De, Salvadori, Laura, Grassia, Rosa, Leone, Carlo A, Rosa, Giuseppe De, Maiuri, Maria C, Pettoello-Mantovani, Massimo, Guido, Stefano, Bossi, Anna, Zolin, Anna, Venerando, Andrea, Pinna, Lorenzo A, Mehta, Anil, Bona, Gianni, Kroemer, Guido, Maiuri, Luigi, Raia, Valeria
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Taylor & Francis 2014
Materias:
Translational Research Papers
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4502695/
https://www.ncbi.nlm.nih.gov/pubmed/25350163
http://dx.doi.org/10.4161/15548627.2014.973737

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