Subject: химия chem. Copper-containing enzymes include ceruloplasmin, SOD, cytochrome-c oxidase, tyrosinase, monoamine oxidase, lysyl oxidase and phenylalanine hydroxylase. В контексте Copper is required for the proper functioning of many important enzyme systems. Copper-containing enzymes include ceruloplasmin, SOD, cytochrome-c oxidase, tyrosinase, monoamine oxidase, lysyl oxidase and phenylalanine hydroxylase. Wilson disease is an autosomal recessive disorder that Leads to Copper toxicity, because Copper accumulates in the liver, brain and eyes. Wilson disease affects the hepatic intracellular transport of Copper and its subsequent inclusion into ceruloplasmin and bile. Because Copper is not incorporated into ceruloplasmin, its normal systemic distribution is impaired, and Copper accumulates in the liver, brain and eyes. Wilson disease generally appears in late childhood and is accompanied by hepatic cirrhosis, neurological degeneration and Copper deposits in the cornea of the eye. Patients with Wilson disease are treated with chelating agents, such as penicillamine, to promote Copper excretion.
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