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Article

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

Autophagy - 1 Jan 2014

De Stefano Daniela, Villella Valeria R, Esposito Speranza, Tosco Antonella, Sepe Angela, De Gregorio Fabiola, Salvadori Laura, Grassia Rosa, Leone Carlo A, De Rosa Giuseppe, 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

Abstract excerpt

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 mo...

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Source

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Identifiers

PMID: 25350163
DOI: 10.4161/15548627.2014.973737
PMCID: PMC4502695

Genes

CFTR
TNF
BECN1
CXCL8
SQSTM1

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026. Prepared record publication-25350163-2026-09-04.2. Manifest publication-graph-cohort-2026-09-04.2.

Source receipt: PubMed via Helena Literature Corpus, retrieved 4 Sept 2026.

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