期刊
BIOCHIMICA ET BIOPHYSICA ACTA-MOLECULAR CELL RESEARCH
卷 1783, 期 9, 页码 1585-1594出版社
ELSEVIER SCIENCE BV
DOI: 10.1016/j.bbamcr.2008.04.002
关键词
calnexin; calnexin knockout; CFTR; ER quality control
Cystic fibrosis (CF) is caused by the mutation in CF transmembrane conductance regulator (CFTR), a cAMP-dependent Cl- channel at the plasma membrane of epithelium. The most common mutant, Delta F508 CFTR, has competent Cl- channel function, but fails to express at the plasma membrane since it is retained in the endoplasmic reticulum (ER) by the ER quality control system. Here, we show that calnexin (CNX) is not necessary for the ER retention of Delta F508 CFTR. Our data show that CNX knockout (KO) does not affect the biosynthetic processing, cellular localization or the Cl- channel function of Delta F508 CFTR. Importantly, cAMP induced Cl- current in colonic epithelium from CNX KO/Delta F508 CFFR mice was comparable with that of Delta F508 CFFR mice, indicating that CNX KO failed to rescue the ER retention of Delta F508 CFTR in vivo. Moreover, we show that CNX assures the efficient expression of WT CFFR, but not Delta F508 CFTR, by inhibiting the proteasomal degradation, indicating that CNX might stimulate the productive folding of WT CFFR, but not Delta F508 CFTR, which has folding defects. (c) 2008 Elsevier B.V. All rights reserved.
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