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[78] The process of mitochondrial apo‐SOD1 import and retention is regulated by CCS, translocase of the outer mitochondrial membrane and the Mia40/Erv1 disulfide relay system (Figure 6).[ 76 , 79 ] Immature SOD1 and CCS are translocated from the cytosol into the mitochondrial intermembrane space by translocase of the outer mitochondrial membrane, where they both become trapped upon metal insertion and disulfide bond formation

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doi: 10.1016/j.tifs.2023.06.013 350 MobiniRTremaroliVStåhlmanMKarlssonFLevinMLjungbergMet al