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Article

Disease-relevant proteostasis regulation of cystic fibrosis transmembrane conductance regulator.

Cell death and differentiation - 1 Aug 2013

Villella V R, Esposito S, Bruscia E M, Vicinanza M, Cenci S, Guido S, Pettoello-Mantovani M, Carnuccio R, De Matteis M A, Luini A, Maiuri M C, Raia V, Kroemer G, Maiuri L

Abstract excerpt

Mismanaged protein trafficking by the proteostasis network contributes to several conformational diseases, including cystic fibrosis, the most frequent lethal inherited disease in Caucasians. Proteostasis regulators, as cystamine, enable the beneficial action of cystic fibrosis transmembrane conductance regulator (CFTR) potentiators in ΔF508-CFTR airways beyond drug washout. Here we tested the hypothesis that...

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Identifiers

PMID: 23686137
DOI: 10.1038/cdd.2013.46
PMCID: PMC3705602

Genes

CFTR
SQSTM1
BECN1

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