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Toxics 2014, 2(2), 327-345; doi:10.3390/toxics2020327

Abnormal Copper Homeostasis: Mechanisms and Roles in Neurodegeneration

FNRS—ULB, Unité d'Etude du Mouvement, 808 Route de Lennik, 1070 Bruxelles, Belgium
Received: 6 May 2014 / Revised: 4 June 2014 / Accepted: 5 June 2014 / Published: 18 June 2014
(This article belongs to the Collection Heavy Metals Toxicology)
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As a cofactor of proteins and enzymes involved in critical molecular pathways in mammals and low eukaryotes, copper is a transition metal essential for life. The intra-cellular and extra-cellular metabolism of copper is under tight control, in order to maintain free copper concentrations at very low levels. Copper is a critical element for major neuronal functions, and the central nervous system is a major target of disorders of copper metabolism. Both the accumulation of copper and copper deficiency are associated with brain dysfunction. The redox capacities of free copper, its ability to trigger the production of reactive oxygen species and the close relationships with the regulation of iron and zinc are remarkable features. Major advances in our understanding of the relationships between copper, neuronal functions and neurodegeneration have occurred these last two decades. The metabolism of copper and the current knowledge on the consequences of copper dysregulation on brain disorders are reviewed, with a focus on neurodegenerative diseases, such as Wilson’s disease, Alzheimer’s disease and Parkinson’s disease. In vitro studies, in vivo experiments and evidence from clinical observations of the neurotoxic effects of copper provide the basis for future therapies targeting copper homeostasis.
Keywords: copper; neurotoxicity; ceruloplasmin; cuproenzymes; chaperones; neurodegeneration; brain; Wilson’s disease; Alzheimer’s disease; Parkinson’s disease copper; neurotoxicity; ceruloplasmin; cuproenzymes; chaperones; neurodegeneration; brain; Wilson’s disease; Alzheimer’s disease; Parkinson’s disease
This is an open access article distributed under the Creative Commons Attribution License (CC BY 3.0).

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Manto, M. Abnormal Copper Homeostasis: Mechanisms and Roles in Neurodegeneration. Toxics 2014, 2, 327-345.

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