FIGURE 2.

Mutations within the HM-loop inhibit the ATP7A dephosphorylation without affecting phosphorylation. A, the region of the HM-loop mutated in full-length ATP7A; the residues replaced in ATP7A-Mut1 and Mut2 are shown in bold. B, phosphorylation of Mut1 and Mut2 over time where densitometry data for three independent experiments were averaged; phosphorylation at 2 min is taken as 100%. For copper depletion, protein was pretreated with 200 μm BCS before phosphorylation. C, autoradiography of gels showing changes in protein phosphorylation for WT ATP7A (MNK) and both mutants; Mut1 and Mut2 dephosphorylate more slowly than wild-type ATP7A. D, densitometry data for three independent experiments; the initial level of phosphorylation is taken as 100%. Error bars indicate S.E.