[HTML][HTML] Degradation of a mutant secretory protein, α1-antitrypsin Z, in the endoplasmic reticulum requires proteasome activity

D Qu, JH Teckman, S Omura, DH Perlmutter - Journal of Biological …, 1996 - ASBMB
Degradation of proteins that are retained in the quality control apparatus of the endoplasmic
reticulum (ER) has been attributed to a third proteolytic system, distinct from the lysosomal
and the cytoplasmic ubiquitin-dependent proteosomal proteolytic pathways. However,
several recent studies have shown that ER degradation of a mutant membrane protein,
CFTRΔF508, is at least in part mediated from the cytoplasmic side by the 26 S proteasome.
In this study, we examined the possibility that ER degradation of mutant secretory protein α 1 …