The molecular basis of copper homeostasis and copper-related disorders

被引:117
作者
Llanos, RM [1 ]
Mercer, JFB [1 ]
机构
[1] Deakin Univ, Sch Biol & Chem Sci, Ctr Cellular & Mol Biol, Burwood, Vic 3125, Australia
关键词
D O I
10.1089/104454902753759681
中图分类号
Q5 [生物化学]; Q7 [分子生物学];
学科分类号
071010 ; 081704 ;
摘要
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.
引用
收藏
页码:259 / 270
页数:12
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