Ontology highlight
ABSTRACT:
SUBMITTER: Miyata T
PROVIDER: S-EPMC7578753 | biostudies-literature | 2020 Oct
REPOSITORIES: biostudies-literature
iScience 20201007 10
Misfolded or unfolded proteins in the ER are said to be degraded only after translocation or isolation from the ER. Here, we describe a mechanism by which mutant proteins are degraded within the ER. Aggregates of mutant arginine vasopressin (AVP) precursor were confined to ER-associated compartments (ERACs) connected to the ER in AVP neurons of a mouse model of familial neurohypophysial diabetes insipidus. The ERACs were enclosed by membranes, an ER chaperone and marker protein of phagophores an ...[more]