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The role of copper in neurodegenerative disease.
Neurobiol Dis. 1999 Aug; 6(4):221-30.ND

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.

Authors+Show Affiliations

Edward Mallinckrodt Department of Pediatrics, Washington University School of Medicine, St. Louis, Missouri, USA.No affiliation info availableNo affiliation info available

Pub Type(s)

Journal Article
Research Support, Non-U.S. Gov't
Research Support, U.S. Gov't, P.H.S.
Review

Language

eng

PubMed ID

10448050

Citation

Waggoner, D J., et al. "The Role of Copper in Neurodegenerative Disease." Neurobiology of Disease, vol. 6, no. 4, 1999, pp. 221-30.
Waggoner DJ, Bartnikas TB, Gitlin JD. The role of copper in neurodegenerative disease. Neurobiol Dis. 1999;6(4):221-30.
Waggoner, D. J., Bartnikas, T. B., & Gitlin, J. D. (1999). The role of copper in neurodegenerative disease. Neurobiology of Disease, 6(4), 221-30.
Waggoner DJ, Bartnikas TB, Gitlin JD. The Role of Copper in Neurodegenerative Disease. Neurobiol Dis. 1999;6(4):221-30. PubMed PMID: 10448050.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - The role of copper in neurodegenerative disease. AU - Waggoner,D J, AU - Bartnikas,T B, AU - Gitlin,J D, PY - 1999/8/17/pubmed PY - 1999/8/17/medline PY - 1999/8/17/entrez SP - 221 EP - 30 JF - Neurobiology of disease JO - Neurobiol. Dis. VL - 6 IS - 4 N2 - 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. SN - 0969-9961 UR - https://www.unboundmedicine.com/medline/citation/10448050/The_role_of_copper_in_neurodegenerative_disease_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0969-9961(99)90250-4 DB - PRIME DP - Unbound Medicine ER -