Participation of the endoplasmic reticulum chaperone calnexin (p88, IP90) in the biogenesis of the cystic fibrosis transmembrane conductance regulator.

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 as abnormal by the cellular "quality control" machinery and is retained within the endoplasmic reticulum (ER). We have...

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