Article
Human copper-transporting ATPase ATP7B (the Wilson's disease protein): biochemical properties and regulation.
Journal of bioenergetics and biomembranes - 1 Oct 2002
Lutsenko Svetlana, Efremov Roman G, Tsivkovskii Ruslan, Walker Joel M
Abstract excerpt
Wilson's disease protein (WNDP) is a product of a gene ATP7B that is mutated in patients with Wilson's disease, a severe genetic disorder with hepatic and neurological manifestations caused by accumulation of copper in the liver and brain. In a cell, WNDP transports copper across various cell membranes using energy of ATP-hydrolysis. Copper regulates WNDP at several levels, modulating its catalytic activity,...
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