Article
Failure of cAMP agonists to activate rescued deltaF508 CFTR in CFBE41o- airway epithelial monolayers.
The Journal of physiology - 1 Dec 2005
Bebok Zsuzsa, Collawn James F, Wakefield John, Parker William, Li Yao, Varga Karoly, Sorscher Eric J, Clancy J P
Abstract excerpt
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, deltaF508, results in endoplasmic reticulum-associated degradation (ERAD) of CFTR. DeltaF508 CFTR has been described as a temperature-sensitive mutation that can be rescued following growth at 27 degrees C. In order to study...
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