Critical roles of protein disulfide isomerases in balancing proteostasis in the nervous system.

ABSTRACT: Protein disulfide isomerases (PDIs) constitute a family of oxidoreductases promoting redox protein folding and quality control in the endoplasmic reticulum. PDIs catalyze disulfide bond formation, isomerization, and reduction, operating in concert with molecular chaperones to fold secretory cargoes in addition to directing misfolded proteins to be refolded or degraded. Importantly, PDIs are emerging as key components of the proteostasis network, integrating protein folding status with central surveillance mechanisms to balance proteome stability according to cellular needs. Recent advances in the field driven by the generation of new mouse models, human genetic studies, and omics methodologies, in addition to interventions using small molecules and gene therapy, have revealed the significa

SUBMITTER: Medinas DB 

PROVIDER: S-EPMC9253707 | biostudies-literature | 2022 Jul

REPOSITORIES: biostudies-literature