Mutation-Specific Potency and Efficacy of Cystic Fibrosis Transmembrane Conductance Regulator Chloride Channel Potentiators

Cystic fibrosis (CF) is caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) Cl(-) channel. The mutations G551D and G1349D, which affect the nucleotide-binding domains (NBDs) of CFTR protein, reduce channel activity. This defect can be corrected pharmacologically by...

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Bibliografiska uppgifter
Huvudupphovsmän: Caputo, Antonella, Hinzpeter, Alexandre, Caci, Emanuela, Pedemonte, Nicoletta, Arous, Nicole, Di Duca, Marco, Zegarra-Moran, Olga, Fanen, Pascale, Galietta, Luis J. V.
Materialtyp: Artigo
Språk:Inglês
Publicerad: American Society for Pharmacology and Experimental Therapeutics 2009
Ämnen:
Cellular and Molecular
Länkar:https://ncbi.nlm.nih.gov/pmc/articles/PMC2729795/
https://ncbi.nlm.nih.gov/pubmed/19491324
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1124/jpet.109.154146
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