Some gating potentiators, including VX-770, diminish ΔF508-CFTR functional expression

Cystic fibrosis (CF) is caused by mutations in the CF transmembrane regulator (CFTR) that result in reduced anion conductance at the apical membrane of secretory epithelia. Treatment of CF patients carrying the G551D gating mutation with the potentiator VX-770 (ivacaftor) largely restores channel ac...

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Udgivet i:Sci Transl Med
Main Authors: Veit, Guido, Avramescu, Radu G., Perdomo, Doranda, Phuan, Puay-Wah, Bagdany, Miklos, Apaja, Pirjo M., Borot, Florence, Szollosi, Daniel, Wu, Yu-Sheng, Finkbeiner, Walter E., Hegedus, Tamas, Verkman, Alan S., Lukacs, Gergely L.
Format: Artigo
Sprog:Inglês
Udgivet: 2014
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC4467693/
https://ncbi.nlm.nih.gov/pubmed/25101887
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/scitranslmed.3008889
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