Cyanoquinolines with Independent Corrector and Potentiator Activities Restore ΔPhe508-Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Function in Cystic Fibrosis

The ΔPhe508 mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) protein impairs its folding, stability, and chloride channel gating. Although small molecules that separately correct defective ΔPhe508-CFTR folding/cellular processing (“correctors”) or chloride channel gating (“...

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Main Authors: Phuan, Puay-Wah, Yang, Baoxue, Knapp, John M., Wood, Alex B., Lukacs, Gergely L., Kurth, Mark J., Verkman, A. S.
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Pharmacology and Experimental Therapeutics 2011
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Articles
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC3187530/
https://ncbi.nlm.nih.gov/pubmed/21730204
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1124/mol.111.073056
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