Article
Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling.
British journal of pharmacology - 1 May 2013
Monterisi Stefania, Casavola Valeria, Zaccolo Manuela
Abstract excerpt
The cystic fibrosis conductance regulator (CFTR) is a cAMP-regulated Cl(-) channel expressed predominantly at the apical membrane of secreting epithelial cells. Mutations in the CFTR gene lead to cystic fibrosis, the most frequent genetic disease in the Caucasian population. The most common mutation, a deletion of phenylalanine at position 508 (F508del), impairs CFTR folding and chloride channel function....
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