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Quality Control in the ER: Misfolded Prohormones Get a Checkup
- Sprunger, Macy L., Jackrel, Meredith E.
- Molecular cell 2019 v.75 no.3 pp. 415-416
- diabetes, endoplasmic reticulum, insulin secretion, mutants, proinsulin, quality control, youth
- In Mutant INS-gene-induced Diabetes of Youth (MIDY) syndrome, mutant proinsulin aggregates interfere with the folding of wild-type proinsulin in the endoplasmic reticulum, ultimately decreasing insulin secretion. In this issue of Molecular Cell, Cunningham et al. (2019) identify two mechanisms by which prohormone aggregation is prevented and cleared.