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Papers In Press, published online ahead of print August 30, 2005
Department of Physiology, Dartmouth Medical School, Hanover, NH 03755
Corresponding Author: Agnieszka.Swiatecka-Urban{at}Dartmouth.edu
The most common mutation in the CFTR gene in individuals with cystic fibrosis, F508, causes retention of F508-CFTR in the endoplasmic reticulum and leads to the absence of CFTR Cl- channels in the apical plasma membrane. Rescue of F508-CFTR, by reduced temperature or chemical means, reveals that the F508 mutation reduces the half-life of F508-CFTR in the apical plasma membrane. Because F508-CFTR retains some Cl- channel activity, increased expression of F508-CFTR in the apical membrane could serve as a potential therapeutic approach for cystic fibrosis. However, little is known about the mechanisms responsible for the short apical membrane half-life of F508-CFTR in polarized human airway epithelial cells. Accordingly, the goal of this study was to determine the cellular defects in the trafficking of rescued F508-CFTR that lead to the decreased apical membrane half-life of F508-CFTR in polarized human airway epithelial cells. We report that in polarized human airway epithelial cells (CFBE41o-), the F508 mutation increased endocytosis of CFTR from the apical membrane without causing a global endocytic defect or affecting the endocytic recycling of CFTR in the Rab11a-specific apical recycling compartment.
J. Biol. Chem, 10.1074/jbc.M508944200
Submitted on August 12, 2005
Revised on August 29, 2005
Accepted on August 30, 2005
The short apical membrane half-life of rescued ùF508-CFTR results from accelerated endocytosis ùF508-CFTR in polarized human airway epithelial cells
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