Chaperone displacement from mutant cystic fibrosis transmembrane conductance regulator restores its function in human airway epithelia

Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene cause cystic fibrosis (CF). The most common mutation, ΔF508, omits the phenylalanine residue at position 508 in the first nucleotide binding domain (NBD1) of CFTR. The mutant protein is retained in the endoplasmic retic...

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Autori principali: Sun, Fei, Mi, Zhibao, Condliffe, Steven B., Bertrand, Carol A., Gong, Xiaoyan, Lu, Xiaoli, Zhang, Ruilin, Latoche, Joseph D., Pilewski, Joseph M., Robbins, Paul D., Frizzell, Raymond A.
Natura: Artigo
Lingua:Inglês
Pubblicazione: The Federation of American Societies for Experimental Biology 2008
Soggetti:
Research Communications
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC2518260/
https://ncbi.nlm.nih.gov/pubmed/18556464
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1096/fj.07-105338
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