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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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Detalles Bibliográficos
Autores principales: Lopes-Pacheco, Miquéias, Boinot, Clément, Sabirzhanova, Inna, Rapino, Daniele, Cebotaru, Liudmila
Formato: Online Artículo Texto
Lenguaje:English
Publicado: 2017
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7082854/
https://www.ncbi.nlm.nih.gov/pubmed/28448979
http://dx.doi.org/10.1159/000475578