The Link between Copper and Wilson's Disease

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.

Copper toxicology, oxidative stress and inflammation using zebrafish as experimental model

“…Acquired copper deficiency is less frequent in developed countries because copper is present in numerous foods; however, it has been documented in long‐term parenteral nutrition patients (Woinmant and Trocello, ), and as a consequence of zinc excess from some dental adhesives (Hedera et al , ). Non‐genetic copper toxicosis is also exceptional, and related to accidental exposure, ingestion of copper‐contaminated food or drinking water or deliberate ingestion of Cu 2 + salts (Purchase, ). Pathophysiological processes including tumor growth, cancer resistance and oxidative stress that contribute to inflammation, cardiovascular and neurodegenerative diseases, have been associated with a copper imbalance (La Fontaine et al ., ).…”

Determination of Heavy Metal Concentrations in Normal and Pathological Human Endometrial Biopsies and In Vitro Regulation of Gene Expression by Metals in the Ishikawa and Hec-1b Endometrial Cell Line

“…Depending upon the metal and its uses, human exposure may vary. The central nervous system is the main target organ that has been identified for the deleterious effects of toxic metals [ 3 – 6 ]. Little data exist concerning the accumulation of metals in other organs, including the reproductive organs, particularly in women, despite the fact that several heavy metals are described as endocrine disruptors [ 7 ].…”

Sir John Cornforth Ac Cbe Frs: His Synthetic Work

“…The structure of D-penicillamine accounted for a significant portion of the core of the penicillins. Subsequently D-penicillamine has been shown to have a number of other important therapeutic properties including the treatment of Wilson's disease 19,20 , lead poisoning and as an anti-rheumatic drug.…”