Article
Mutation-specific potency and efficacy of cystic fibrosis transmembrane conductance regulator chloride channel potentiators.
The Journal of pharmacology and experimental therapeutics - 1 Sept 2009
Caputo Antonella, Hinzpeter Alexandre, Caci Emanuela, Pedemonte Nicoletta, Arous Nicole, Di Duca Marco, Zegarra-Moran Olga, Fanen Pascale, Galietta Luis J V
Abstract excerpt
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 small molecules called potentiators. CF mutations residing in the intracellular loops (ICLs), connecting the...
Read the complete abstract on PubMedTopics
Share this publication in a Topic to start or enrich a Post.