The link between copper and Wilson’s disease

Purchase, Rupert (2013) The link between copper and Wilson’s disease. Science Progress, 96 (3). pp. 213-223. ISSN 0036-8504

[img] PDF - Published Version
Restricted to SRO admin only

Download (525kB)

Abstract

Wilson’s disease (hepatolenticular degeneration) is a rare inherited autosomal recessive disorder of copper metabolism leading to copper accumulation in the liver and extrahepatic organs such as the brain and cornea. Patients may present with combinations of hepatic, neurological and psychiatric symptoms. Copper is the therapeutic target for the treatment of Wilson’s disease. But how did copper come to be linked with Wilson’s disease? The answer encompasses a study of enzootic neonatal ataxia in lambs in the 1930s, the copper-chelating properties of British Anti-Lewisite, and the chemical analysis for copper of the organs of deceased Wilson’s disease patients in the mid-to-late 1940s. Wilson’s disease is one of a number of copper-related disorders where loss of copper homeostasis as a result of genetic, nutritional or environmental factors affects human health.

Item Type: Article
Keywords: Hepatolenticular degeneration, Wilson’s disease, copper metabolism, British Anti-Lewisite, BAL, BAL-Intrav, D-penicillamine, trientine dihydrochloride, zinc sulfate, zinc acetate, ammonium tetrathiomolybdate, enzootic ataxia, swayback, demyelinating diseases, Menkes disease, copper deficiency myelopathy.
Schools and Departments: School of Life Sciences > Chemistry
Depositing User: Rupert Purchase
Date Deposited: 11 Dec 2017 15:00
Last Modified: 11 Dec 2017 15:00
URI: http://sro.sussex.ac.uk/id/eprint/71967

View download statistics for this item

📧 Request an update