Human pancreatic islets and pancreas tissue were isolated from deceased donors under ethical approval from the Human Research Ethics Board of the University of Alberta (Pro00013094, Pro00001754) and obtained from the NIDDK-funded Integrated Islet Distribution Program (IIDP) (RRID: SCR _014387)
BCKAs can be metabolized by BCKDH to branched-chain acyl-CoA, and blocking this step causes serious metabolic disorders, such as maple syrup urine disease (Xu et al., 2020) and propionic academia (Chen et al., 2017)
doi:10.1056/NEJMoa1603827 Marso SP, Bain SC, Consoli A, et al
10.1038/nrn2038 11 BottinoF.LucignaniM.NapolitanoA.DellepianeF.ViscontiE.Rossi EspagnetM
Again, this impairment is effectively bypassed by supplementation with gamma-glutamylcysteine, which can be transported into cells where it will be rapidly converted to GSH by GSH synthase