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Metals are indispensable elements of cell biology. They function as cofactors in many specific proteins and are involved in all major metabolic pathways. Their metabolism and implications in inborn errors of metabolism are still not well known, but the number of inherited metabolic disorders involving the absorption, transport, or metabolism of metals is rapidly growing. Clinical presentations are very diverse and can involve all organs and systems, including the liver and the central nervous system (Table 169-1). Deficiency in metals results in metabolic abnormalities due mostly to loss of function of metal-dependent proteins. On the other hand, excess of metals can result in the unregulated oxidation of proteins, lipids, and other cellular components, causing subsequent tissue injury. Some inherited metal disorders are treatable by chelating drugs or by daily supplementation of the missing metal at pharmacological doses.

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Table 169-1. Inborn Errors of Metal Transport and Metabolism

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