Calnexin Δ185–520 partially reverses the misprocessing of the ΔF508 cystic fibrosis transmembrane conductance regulator1
- 8 August 2002
- journal article
- Published by Wiley in FEBS Letters
- Vol. 526 (1-3) , 87-92
- https://doi.org/10.1016/s0014-5793(02)03134-4
Abstract
Abnormal retention of ΔF508 CFTR (cystic fibrosis transmembrane conductance regulator) in the endoplasmic reticulum is a major cause of cystic fibrosis (CF). We show that calnexin Δ185–520 but not calnexin can partially reverse the mislocalization of ΔF508 CFTR. This 256‐amino acid protein has neither the transmembrane domain nor the P domain of calnexin. Calnexin Δ185–520 interacted with CFTR directly, and was secreted into the extracellular compartment over time. Forty‐eight hours after transfection into CHO cells, calnexin Δ185–520 increased the conversion of immature ΔF508 CFTR into mature ΔF508 CFTR. In immortalized human CF cell lines expressing ΔF508 CFTR, a halide efflux assay showed that calnexin Δ185–520 partially restored CFTR function. These data indicate that calnexin Δ185–520 may give a clue to develop the therapeutic way of cystic fibrosis with ΔF508 CFTR.Keywords
This publication has 37 references indexed in Scilit:
- Setting the Standards: Quality Control in the Secretory PathwayScience, 1999
- Glycerol Reverses the Misfolding Phenotype of the Most Common Cystic Fibrosis MutationJournal of Biological Chemistry, 1996
- Purification and functional reconstitution of the cystic fibrosis transmembrane conductance regulator (CFTR)Cell, 1992
- The spectrum of cystic fibrosis mutationsTrends in Genetics, 1992
- Chloride Conductance Expressed by ΔF508 and Other Mutant CFTRs In Xenopus OocytesScience, 1991
- Naturally occurring mutations in intestinal sucrase-isomaltase provide evidence for the existence of an intracellular sorting signal in the isomaltase subunit [published erratum appears in J Cell Biol 1991 Dec;115(5):following 1473]The Journal of cell biology, 1991
- Phosphorylation-regulated CI− channel in CHO cells stably expressing the cystic fibrosis geneNature, 1991
- Identification of the Cystic Fibrosis Gene: Chromosome Walking and JumpingScience, 1989
- Identification of the Cystic Fibrosis Gene: Genetic AnalysisScience, 1989
- Identification of the Cystic Fibrosis Gene: Cloning and Characterization of Complementary DNAScience, 1989