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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Bibliografiska uppgifter
I publikationen:Sci Transl Med
Huvudupphovsmän: 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.
Materialtyp: Artigo
Språk:Inglês
Publicerad: 2014
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Article
Länkar: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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