The molecular basis of copper homeostasis and copper-related disorders

Llanos, Roxana and Mercer, Julian 2002, The molecular basis of copper homeostasis and copper-related disorders, DNA and cell biology, vol. 21, no. 4, pp. 259-270, doi: 10.1089/104454902753759681.

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Title The molecular basis of copper homeostasis and copper-related disorders
Author(s) Llanos, Roxana
Mercer, Julian
Journal name DNA and cell biology
Volume number 21
Issue number 4
Start page 259
End page 270
Publisher Mary Ann Liebert Inc. Publishers
Place of publication New York, N.Y.
Publication date 2002-04-01
ISSN 1044-5498
Summary Copper is an essential trace element that can be extremely toxic in excess due to the pro-oxidant activity of copper ions. Inherited disorders of copper transport, Menkes disease (copper deficiency), and Wilson disease (copper toxicosis) are caused by mutations of two closely related Cu transporting-ATPases, and demonstrate the essentiality and potential toxicity of copper. Other copper toxicosis conditions in humans and animals have been described, but are not well understood at a molecular level. Copper homeostatic mechanisms are being discovered. One such mechanism is copper-induced trafficking of the Cu-ATPases, which allows cells to provide copper to secreted cupro-proteins but also to efflux excess copper. Oxidative damage induced by copper may be involved in the pathogenesis of neurodegenerative conditions such as Alzheimer's disease, familial amyotrophic lateral sclerosis, and prion diseases.
Language eng
DOI 10.1089/104454902753759681
Field of Research 060499 Genetics not elsewhere classified
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2002, Mary Ann Liebert Publishers
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