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

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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Bibliographic Details
Main Authors: Miquéias Lopes-Pacheco, Clément Boinot, Inna Sabirzhanova, Daniele Rapino, Liudmila Cebotaru
Format: Article
Language:English
Published: Cell Physiol Biochem Press GmbH & Co KG 2017-04-01
Series:Cellular Physiology and Biochemistry
Subjects:
CFTR mutants
Cystic fibrosis
Electrophysiology
Heat shock protein
Intracellular trafficking
Membrane-spanning
Precision medicine
Protein folding
Proteostasis network
Online Access:http://www.karger.com/Article/FullText/475578

Internet

http://www.karger.com/Article/FullText/475578

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