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Potentiator Ivacaftor Abrogates Pharmacological Correction of ΔF508 CFTR in Cystic Fibrosis

Cystic Fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator (CFTR). Newly developed “correctors” such as lumacaftor (VX-809) that improve CFTR maturation and trafficking and “potentiators” such as ivacaftor (VX-770) that enhance channel activity may provide important ad...

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Publicat a:Sci Transl Med
Autors principals: Cholon, Deborah M., Quinney, Nancy L., Fulcher, M. Leslie, Esther, Charles R., Das, Jhuma, Dokholyan, Nikolay V., Randell, Scott H., Boucher, Richard C., Gentzsch, Martina
Format: Artigo
Idioma:Inglês
Publicat: 2014
Matèries:
Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC4272825/
https://ncbi.nlm.nih.gov/pubmed/25101886
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1126/scitranslmed.3008680
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