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Functional Stability of Rescued ΔF508 Cystic Fibrosis Transmembrane Conductance Regulator in Airway Epithelial Cells

The most common mutation in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) gene, ΔF508, results in the production of a misfolded protein that is rapidly degraded. The mutant protein is temperature sensitive, and prior studies indicate that the low-temperature–rescued channel is...

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Главные авторы: Jurkuvenaite, Asta, Chen, Lan, Bartoszewski, Rafal, Goldstein, Rebecca, Bebok, Zsuzsa, Matalon, Sadis, Collawn, James F.
Формат: Artigo
Язык:Inglês
Опубликовано: American Thoracic Society 2010
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Online-ссылка:https://ncbi.nlm.nih.gov/pmc/articles/PMC2830406/
https://ncbi.nlm.nih.gov/pubmed/19502384
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1165/rcmb.2008-0434OC
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