Hassan A, Masood F.
Wilson`s disease: a review.
J Pak Med Assoc Jan ;54(9):479-84.

Wilson`s disease is a disease of copper toxicity. Absorbed dietary copper is bound mainly to albumin in the portal circulation from which it is avidly extracted by hepatocytes. Hepatocellular copper is subsequently used for cellular metabolic needs, incorporated into ceruloplasmin or excreted into bile. The transport of hepatocellular copper to bile is thought to involve a vesicular pathway (Golgi apparatus) that depends on ATP7B (copper transporting Ptype ATPase) function. The absence or diminished function of ATP7B results in a decrease in biliary copper excretion, which is responsible for the hepatic accumulation of this metal in Wilson`s disease. This is a review article.

PakMediNet -Pakistan's largest Database of Pakistani Medical Journals - http://www.pakmedinet.com