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Synergy-Based Small-Molecule Screen Using a Human Lung Epithelial Cell Line Yields ΔF508-CFTR Correctors That Augment VX-809 Maximal Efficacy

The most prevalent cystic fibrosis transmembrane conductance regulator (CFTR) mutation causing cystic fibrosis, ΔF508, impairs folding of nucleotide binding domain (NBD) 1 and stability of the interface between NBD1 and the membrane-spanning domains. The interfacial stability defect can be partially...

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Detalhes bibliográficos
Main Authors: Phuan, Puay-Wah, Veit, Guido, Tan, Joseph, Roldan, Ariel, Finkbeiner, Walter E., L. Lukacs, Gergely, Verkman, A. S.
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society for Pharmacology and Experimental Therapeutics 2014
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC4054004/
https://ncbi.nlm.nih.gov/pubmed/24737137
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1124/mol.114.092478
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