Article
The short apical membrane half-life of rescued {Delta}F508-cystic fibrosis transmembrane conductance regulator (CFTR) results from accelerated endocytosis of {Delta}F508-CFTR in polarized human airway epithelial cells.
The Journal of biological chemistry - 4 Nov 2005
Swiatecka-Urban Agnieszka, Brown Andrea, Moreau-Marquis Sophie, Renuka Janhavi, Coutermarsh Bonita, Barnaby Roxanna, Karlson Katherine H, Flotte Terence R, Fukuda Mitsunori, Langford George M, Stanton Bruce A
Abstract excerpt
The most common mutation in the cystic fibrosis transmembrane conductance regulator (CFTR) gene in individuals with cystic fibrosis, DeltaF508, causes retention of DeltaF508-CFTR in the endoplasmic reticulum and leads to the absence of CFTR Cl(-) channels in the apical plasma membrane. Rescue of DeltaF508-CFTR by reduced temperature or chemical means reveals that the DeltaF508 mutation reduces the half-life of...
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