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Prog Neurobiol. 2014 May;116:33-57. doi: 10.1016/j.pneurobio.2014.01.002. Epub 2014 Jan 17.

Metabolism and functions of copper in brain.

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Department of Parasitology, Faculty of Science, Charles University, Prague, Czech Republic.
Centre for Cellular and Molecular Biology, School of Life and Environmental Sciences, Deakin University, Burwood Campus, 221 Burwood Highway, Burwood, Victoria 3125, Australia.
Center for Biomolecular Interactions Bremen, University of Bremen, P.O. Box 330440, D-28334 Bremen, Germany; Center for Environmental Research and Sustainable Technology, Leobener Strasse, D-28359 Bremen, Germany. Electronic address:


Copper is an important trace element that is required for essential enzymes. However, due to its redox activity, copper can also lead to the generation of toxic reactive oxygen species. Therefore, cellular uptake, storage as well as export of copper have to be tightly regulated in order to guarantee sufficient copper supply for the synthesis of copper-containing enzymes but also to prevent copper-induced oxidative stress. In brain, copper is of importance for normal development. In addition, both copper deficiency as well as excess of copper can seriously affect brain functions. Therefore, this organ possesses ample mechanisms to regulate its copper metabolism. In brain, astrocytes are considered as important regulators of copper homeostasis. Impairments of homeostatic mechanisms in brain copper metabolism have been associated with neurodegeneration in human disorders such as Menkes disease, Wilson's disease and Alzheimer's disease. This review article will summarize the biological functions of copper in the brain and will describe the current knowledge on the mechanisms involved in copper transport, storage and export of brain cells. The role of copper in diseases that have been connected with disturbances in brain copper homeostasis will also be discussed.


Astrocytes; Brain; Copper; Neurodegeneration; Transport

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