Wilson disease

Wilson disease

or hepatolenticular degeneration

Recessive hereditary defect (see recessiveness) that impairs one's ability to metabolize copper. In affected persons, copper accumulates in the basal ganglia (see ganglion) of the brain (involved in control of movement), causing progressive degeneration; forms a brownish ring at the margin of the cornea of the eye; and is deposited in the liver, gradually leading to cirrhosis. Other symptoms include tremor, lack of coordination, and personality changes. The disease usually appears in the person's teen years or twenties. Early diagnosis and treatment with a high-protein, low-copper diet and a substance to chelate copper can reverse the effects and prevent permanent brain and liver damage.

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Wilson disease protein (also called ATP7B) is an ATPase that transports copper.

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