Systemic and mitochondrial copper transport. Copper from the diet is imported into enterocytes by Ctr1, then exported into the bloodstream by ATP7A. In the bloodstream, the majority of copper is bound to ceruloplasmin. Copper is transported into cells by Ctr1, and across the outer mitochondrial membrane by an unknown mechanism. In the inter-membrane space, copper is bound by Cox17, which delivers copper to Sco1 or Cox11. Sco1-bound copper is delivered to the Cox1 subunit of Cco. Cox11-bound copper is delivered to the Cox2 subunit of Cco. Cu: Copper. Ce: Ceruloplasmin. OM: outer mitochondrial membrane. IS: intermembrane space. IM: inner mitochondrial membrane. Cco: cytochrome oxidase.