Figure 1.

Diagram of ATP7B. The metal-binding domain contains six copper-binding domains (MBD1-6), all with the conserved sequence motif CXXC. The transmembrane channel consisting of eight discontinuous ion channels may contribute to copper transport (Cylinder1-8). The Cys–Pro–Cys (CPC) sequence motif is the key residue that confers metal ion selectively. ATP binds to the N-domain and the SEHPL motif located in the N-domain. The P-domain is the room for phosphorylation of Asp from the sequence DKTGT. The A-domain is the place where the acyl-phosphate gets dephosphorylated. Mutations may occur at any position of the gene, and then may cause the deposition of copper.