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Local modulation of cystic fibrosis conductance regulator: cytoskeleton and compartmentalized cAMP signalling.

Abstract:

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. Although an intense effort is under way to identify compounds that target the F508del CF...

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Publisher copy:
10.1111/bph.12017

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Institution:
University of Oxford
Department:
Oxford, MSD, Physiology Anatomy and Genetics, BHF Centre of Research Excellence
Role:
Author
Journal:
British journal of pharmacology
Volume:
169
Issue:
1
Pages:
1-9
Publication date:
2013-05-05
DOI:
EISSN:
1476-5381
ISSN:
0007-1188
URN:
uuid:c39ec2cd-6f34-45c6-9eff-1385db714e48
Source identifiers:
356286
Local pid:
pubs:356286

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