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The Role of Copper in Neurodegenerative Disease

Open access
Waggoner, Darrel J., Bartnikas, Thomas B., Gitlin, Jonathan D.·1999·Neurobiology of Disease

Abstract

Copper is an essential trace metal which plays a fundamental role in the biochemistry of the human nervous system. Menkes disease and Wilson disease are inherited disorders of copper metabolism and the dramatic neurodegenerative phenotypes of these two diseases underscore the essential nature of copper in nervous system development as well as the toxicity of this metal when neuronal copper homeostasis is perturbed. Ceruloplasmin contains 95% of the copper found in human plasma and inherited loss of this essential ferroxidase is associated with progressive neurodegeneration of the retina and basal ganglia. Gain-of-function mutations in the cytosolic copper enzyme superoxide dismutase result in the motor neuron degeneration of amyotrophic lateral sclerosis and current evidence suggests a direct pathogenic role for copper in this process. Recent studies have also implicated copper in the pathogenesis of neuronal injury in Alzheimer's disease and the prion-mediated encephalopathies, suggesting that further elucidation of the mechanisms of copper trafficking and metabolism within the nervous system will be of direct relevance to our understanding of the pathophysiology and treatment of neurodegenerative disease.

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Metadata

Authors
Waggoner, D. J., Bartnikas, T. B., & Gitlin, J. D.
Venue
Neurobiology of Disease
Publisher
Elsevier BV
Published
1999-08
Volume
6
Issue
4
Pages
221-230
ISSN
0969-9961 (ISSN-L)
OpenAlex
W2019279182
Cited by
863
References
5
FWCI
15.94
Type
journal article
Language
en
Open access
green

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CoChat. (2026). Citation check: The Role of Copper in Neurodegenerative Disease. https://app.cochat.ai/papers/10.1006/nbdi.1999.0250
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Waggoner, D. J., Bartnikas, T. B., & Gitlin, J. D. (1999). The Role of Copper in Neurodegenerative Disease. Neurobiology of Disease. https://doi.org/10.1006/nbdi.1999.0250

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