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. 2021 Aug 24;8:711227. doi: 10.3389/fmolb.2021.711227

FIGURE 2.

FIGURE 2

(A) Schematic of Cu/Zn-SOD1 metalation in CCS1 mitochondria. CuL transfers a copper ion to CCS1 in the intermembrane space. The incorporation of zinc may be during or before metalation with copper by CCS1. SOD1 is metalated in the intermembrane space (IMS) of mitochondria and then enters the matrix (Cobine et al., 2006a; Cobine et al., 2006b; Robinson and Winge 2010; Fukai and Ushio-Fukai 2011). (B) Metalation of COX copper centers (CuA and CuB sites). COX is assembled in parts that are then merged to produce the matured holoenzyme complex. The COX1 subunit contains the CuB site, and the COX2 subunit, the CuA site. After entering mitochondria, CuL donates two copper ions to the copper chaperone COX17 which in turn transfers the copper ions to COX11 and SCO2. COX11 metalates the CuB site of the subunit COX1, and SCO2, the CuA site of subunit COX2 by delivering two copper ions in a SCO1-mediated process (Robinson and Winge 2010; Jett and Leary 2018).