File(s) not publicly available
The link between copper and Wilson’s disease
journal contribution
posted on 2023-06-20, 14:16 authored by Rupert PurchaseWilson’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.
History
Publication status
- Published
File Version
- Published version
Journal
Science ProgressISSN
0036-8504Publisher
Science Reviews 2000External DOI
Issue
3Volume
96Page range
213-223Department affiliated with
- Chemistry Publications
Full text available
- No
Peer reviewed?
- Yes
Legacy Posted Date
2017-12-11First Compliant Deposit (FCD) Date
2017-12-11Usage metrics
Categories
No categories selectedKeywords
Licence
Exports
RefWorks
BibTeX
Ref. manager
Endnote
DataCite
NLM
DC