Article
Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.
The Journal of biological chemistry - 29 Apr 1994
Pind S, Riordan J R, Williams D B
Abstract excerpt
Deletion of phenylalanine at position 508 (delta F508) in the first nucleotide-binding fold of the cystic fibrosis transmembrane conductance regulator (CFTR) is the most common mutation in patients with cystic fibrosis. Although retaining functional Cl- channel activity, this mutant is recognized...
Topics
- Animals
- CHO Cells
- Calcium-Binding Proteins
- Calnexin
- Cell Line
- Cells, Cultured
- Chaperonins
- Cricetinae
- Cystic Fibrosis
- Cystic Fibrosis Transmembrane Conductance Regulator
- Endoplasmic Reticulum
- Humans
- Kinetics
- Membrane Proteins
- Mutation
- Proteins