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Intracellular Copper Transport and ATP7B, the Wilson’s Disease Protein

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Handbook of Copper Pharmacology and Toxicology

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Abstract

Copper is an essential trace metal for all living organisms because this metal serves as a cofactor for activating numerous enzymes critical for homeostasis. However, when copper exceeds the cellular needs, it is toxic through the production of highly reactive hydroxyl radicals that have deleterious effects on cellular components, including destabilization of plasma membranes and lysosomal membranes, disturbance of mitochondrial respiration, depletion of glutathione reserves, and damage of nucleic acids (1). To prevent the accumulation of copper to the toxic level, cells are provided regulatory mechanisms that maintain the balance of intracellular copper.

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Authors
  1. Kunihiko Terada
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  2. Toshihiro Sugiyama
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Editors and Affiliations

  1. The National Health and Environmental Effects Research Laboratory, Research Triangle Park, NC, USA

    Edward J. Massaro

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Terada, K., Sugiyama, T. (2002). Intracellular Copper Transport and ATP7B, the Wilson’s Disease Protein. In: Massaro, E.J. (eds) Handbook of Copper Pharmacology and Toxicology. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-59259-288-3_13

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  • DOI: https://doi.org/10.1007/978-1-59259-288-3_13

  • Publisher Name: Humana Press, Totowa, NJ

  • Print ISBN: 978-1-61737-266-7

  • Online ISBN: 978-1-59259-288-3

  • eBook Packages: Springer Book Archive

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