Article
General trends in the calnexin-dependent expression and pharmacological rescue of clinical CFTR variants.
eLife - 11 Dec 2025
Tedman Austin, Olson John A, Kim Minsoo, Foye Catherine, Jackson JaNise J, McDonald Eli F, McKee Andrew G, Noguera Karen, Kuntz Charles P, Meiler Jens, Oliver Kathryn E, Plate Lars, Schlebach Jonathan P
Abstract excerpt
Cystic fibrosis (CF) is a genetic disease caused by mutations in the cystic fibrosis transmembrane conductance regulator gene (CFTR). Though most people with CF have one or two copies of the ΔF508 mutation, there are hundreds of other distinct CF mutations that vary in their mechanistic effects and response to therapeutics. Endogenous chaperones are known to have divergent effects on the druggability of CF...
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