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The Biochemistry of Copper in Man and Its Role in the Pathogenesis of Wilson’s Disease (Hepatolenticular Degeneration)

  • J. M. Walshe

Abstract

Wilson’s disease is a hereditary metabolic disease. Clinically it is characterized by disturbances of movement, by subacute or chronic liver disease, by abnormalities of renal function and by visible deposits of copper in the cornea, the Kayser Fleischer rings. The biochemical lesion consists of excessive deposition of copper in most tissues but principally the brain, liver and kidneys; in the plasma the concentration of both copper and the copper protein caeruloplasmin are commonly, but not invariably, reduced but the amounts of copper excreted in the urine are increased. The disease, untreated, is invariably fatal.

Keywords

Primary Biliary Cirrhosis Biliary Excretion Liver Uptake Copper Toxicity Copper Protein 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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© Plenum Publishing Company Ltd. 1972

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  • J. M. Walshe

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