Endoplasmic reticulum thiol oxidase deficiency effect on embryonic fibroblasts
Summary:
Analysis of MEFs deficient in ER thiol oxidases (ERO1a, ERO1b) and PRDX4, enzymes that initiate the disulfide relay. Combined loss-of-function mutations compromise the extracellular matrix and interfere with procollagen maturation. Results provide insight into the molecular basis of these defects.