Article
Delta F508 CFTR pool in the endoplasmic reticulum is increased by calnexin overexpression.
Molecular biology of the cell - 1 Feb 2004
Okiyoneda Tsukasa, Harada Kazutsune, Takeya Motohiro, Yamahira Kaori, Wada Ikuo, Shuto Tsuyoshi, Suico Mary Ann, Hashimoto Yasuaki, Kai Hirofumi
Abstract excerpt
The most common cystic fibrosis transmembrane conductance regulator (CFTR) mutant in cystic fibrosis patients, Delta F508 CFTR, is retained in the endoplasmic reticulum (ER) and is consequently degraded by the ubiquitin-proteasome pathway known as ER-associated degradation (ERAD). Because the prolonged interaction of Delta F508 CFTR with calnexin, an ER chaperone, results in the ERAD of Delta F508 CFTR, calnexin...
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