Human–mouse cystic fibrosis transmembrane conductance regulator (CFTR) chimeras identify regions that partially rescue CFTR-ΔF508 processing and alter its gating defect

The ΔF508 mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene is the most common cause of cystic fibrosis. The mutation disrupts biosynthetic processing, reduces channel opening rate, and decreases protein lifetime. In contrast to human CFTR (hCFTR)-ΔF508, mouse CFTR-ΔF50...

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Detaylı Bibliyografya
Asıl Yazarlar: Dong, Qian, Ostedgaard, Lynda S., Rogers, Christopher, Vermeer, Daniel W., Zhang, Yuping, Welsh, Michael J.
Materyal Türü: Artigo
Dil:Inglês
Baskı/Yayın Bilgisi: National Academy of Sciences 2012
Konular:
Biological Sciences
Online Erişim:https://ncbi.nlm.nih.gov/pmc/articles/PMC3271874/
https://ncbi.nlm.nih.gov/pubmed/22210114
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1073/pnas.1120065109
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