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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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Publicat a:Cell Physiol Biochem
Autors principals: Lopes-Pacheco, Miquéias, Boinot, Clément, Sabirzhanova, Inna, Rapino, Daniele, Cebotaru, Liudmila
Format: Artigo
Idioma:Inglês
Publicat: 2017
Matèries:
Accés en línia: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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