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•In Wilson disease, the processes of incorporation of copper into
ceruloplasmin and excretion of excess copper into bile are impaired. The
transport of copper by the copper-transporting P-type ATPase is defective in
Wilson disease secondary to one of several mutations in the ATP7B gene. The
excess copper acts as a promoter of free radical formation and causes
oxidation of lipids and proteins.
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•Organ dysfunction in patients with WD results from inadequate
biliary excretion of copper and subsequent copper deposition, most notably in
the liver and central nervous system.