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

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Biochemical Aspects of Nervous Diseases

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.

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  1. J. M. Walshe
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Editors and Affiliations

  1. The Institute of Neurology, The National Hospitals for Nervous Diseases, Queen Square, London, W.C.1, UK

    J. N. Cumings (Professor of Chemical Pathology) (Professor of Chemical Pathology)

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Walshe, J.M. (1972). The Biochemistry of Copper in Man and Its Role in the Pathogenesis of Wilson’s Disease (Hepatolenticular Degeneration). In: Cumings, J.N. (eds) Biochemical Aspects of Nervous Diseases. Springer, Boston, MA. https://doi.org/10.1007/978-1-4684-1956-6_4

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  • DOI: https://doi.org/10.1007/978-1-4684-1956-6_4

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