Overview of cellular copper metabolism. In neuronal cells, ATP7A is required for maturation of DBH and PAM. ATOX1, copper transport protein ATOX1; ATP7A, copper-transporting ATPase 1; ATP7B, copper-transporting ATPase 2; CCS, copper chaperone for SOD1; CCO, cytochrome c oxidase; COX11, cytochrome c oxidase assembly protein Cox11; Cox17, cytochrome c oxidase copper chaperone; CP, ceruloplasmin; CTR, copper transporter 1; DBH, dopamine-β-hydroxylase; DMT1, divalent metal transporter 1; PAM, peptidylglycine α-amidating monooxygenase; Sco1, protein SCO1 homologue; SOD1, superoxide dismutase. Reproduced from
Kaler (2011), with permission.