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- W98643105 abstract "The current issue of Seminars in Liver Disease is devoted to iron and copper metabolism and related diseases. Iron and copper are transition metals, essential for the survival of most organisms, particularly those existing in an oxygen-rich environment, due to their capacity to participate in one-electron exchange reactions. Their “essentiality” relates both to our need to obtain a supply of these transitional metals from our diet and from their function as critical cofactors in numerous essential proteins: iron in the heme-containing proteins, electron transport chain, and microsomal electron transport proteins; and copper in superoxide dismutase, lysyl oxidase, cytochrome c oxidase, tyrosinase and dopamine-β-hydroxylase. Interestingly, the same property that makes iron and copper essential also may generate noxious free radicals that can cause injury to cell membranes and organelles. Therefore, concentrations of circulating iron and copper demand a concerted and tight regulation as both excess or deficiency impairs cellular functions and causes cell toxicity and organ disease. Excess tissue iron or copper is found in numerous human diseases, and is typified by hereditary hemochromatosis (HH) and Wilson disease (WD), two of the more common genetic disease states associated with deranged metabolism of metals in humans. Recently, we have witnessed dramatic advances in iron and copper biology and learned how their dysregulated metabolism in the liver may lead to severe systemic diseases. The parallel rapid progress in genetics has impacted diagnostic strategies and management of common iron and copper disorders." @default.
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- W98643105 date "2011-08-01" @default.
- W98643105 modified "2023-09-25" @default.
- W98643105 title "Metal Storage Disorders" @default.
- W98643105 doi "https://doi.org/10.1055/s-0031-1286053" @default.
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