[HTML][HTML] Thermally unstable gating of the most common cystic fibrosis mutant channel (ΔF508):“rescue” by suppressor mutations in nucleotide binding domain 1 and …
W Wang, GO Okeyo, B Tao, JS Hong, KL Kirk - Journal of Biological …, 2011 - ASBMB
Most cystic fibrosis (CF) cases are caused by the ΔF508 mutation in the CF transmembrane
conductance regulator (CFTR), which disrupts both the processing and gating of this
chloride channel. The cell surface expression of ΔF508-CFTR can be" rescued" by culturing
cells at 26–28° C and treating cells with small molecule correctors or intragenic suppressor
mutations. Here, we determined whether these various rescue protocols induce a ΔF508-
CFTR conformation that is thermally stable in excised membrane patches. We also tested …
conductance regulator (CFTR), which disrupts both the processing and gating of this
chloride channel. The cell surface expression of ΔF508-CFTR can be" rescued" by culturing
cells at 26–28° C and treating cells with small molecule correctors or intragenic suppressor
mutations. Here, we determined whether these various rescue protocols induce a ΔF508-
CFTR conformation that is thermally stable in excised membrane patches. We also tested …