Failure of cAMP agonists to activate rescued ΔF508 CFTR in CFBE41o(–) airway epithelial monolayers

The cystic fibrosis transmembrane conductance regulator (CFTR) is a cyclic AMP-regulated chloride channel. Mutations in the CFTR gene result in cystic fibrosis (CF). The most common mutation, ΔF508, results in endoplasmic reticulum-associated degradation (ERAD) of CFTR. ΔF508 CFTR has been described...

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Autores principales: Bebok, Zsuzsa, Collawn, James F, Wakefield, John, Parker, William, Li, Yao, Varga, Karoly, Sorscher, Eric J, Clancy, JP
Formato: Artigo
Lenguaje:Inglês
Publicado: Blackwell Science Inc 2005
Materias:
Tissue, System and Organ Physiology
Acceso en línea:https://ncbi.nlm.nih.gov/pmc/articles/PMC1464253/
https://ncbi.nlm.nih.gov/pubmed/16210354
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1113/jphysiol.2005.096669
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