Role of the copper-binding domain in the copper transport function of ATP7B, the P-type ATPase defective in Wilson disease.

Abstract

We have analyzed the functional effect of site-directed mutations and deletions in the copper-binding domain of ATP7B (the copper transporting P-type ATPase defective in Wilson disease) using a yeast complementation assay. We have shown that the sixth copper-binding motif alone is sufficient, but not essential, for normal ATP7B function. The N-terminal two… (More)

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