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Combination of Correctors Rescues CFTR Transmembrane-Domain Mutants by Mitigating their Interactions with Proteostasis

BACKGROUND/AIMS: Premature degradation of mutated cystic fibrosis transmembrane conductance regulator (CFTR) protein causes cystic fibrosis (CF), the commonest Mendelian disease in Caucasians. Despite recent advances in precision medicines for CF patients, many CFTR mutants have not been characteriz...

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Detalhes bibliográficos
Publicado no:Cell Physiol Biochem
Main Authors: Lopes-Pacheco, Miquéias, Boinot, Clément, Sabirzhanova, Inna, Rapino, Daniele, Cebotaru, Liudmila
Formato: Artigo
Idioma:Inglês
Publicado em: 2017
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Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC7082854/
https://ncbi.nlm.nih.gov/pubmed/28448979
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1159/000475578
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