Ontology highlight
ABSTRACT:
SUBMITTER: Farinha CM
PROVIDER: S-EPMC1140594 | biostudies-literature | 2005 Jun
REPOSITORIES: biostudies-literature
Farinha Carlos M CM Amaral Margarida D MD
Molecular and cellular biology 20050601 12
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 regulator (CFTR) with F508del (the most frequent mutation found in patients with th ...[more]