Article
Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Molecular and cellular biology - 1 Jun 2005
Farinha Carlos M, Amaral Margarida D
Abstract excerpt
Biosynthesis and folding of multidomain transmembrane proteins is a complex process. Structural fidelity is monitored by endoplasmic reticulum (ER) quality control involving the molecular chaperone calnexin. Retained misfolded proteins undergo ER-associated degradation (ERAD) through the ubiquitin-proteasome pathway. Our data show that the major degradation pathway of the cystic fibrosis transmembrane conductance...
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