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Copper in disorders with neurological symptoms: Alzheimer`s, Menkes, and Wilson Diseases

Strausak, Daniel, Mercer, Julian, Dieter, Hermann H., Stremmel, Wolfgang and Multhaup, Gerd 2001, Copper in disorders with neurological symptoms: Alzheimer`s, Menkes, and Wilson Diseases, Brain research bulletin, vol. 55, no. 2, pp. 175-185, doi: 10.1016/S0361-9230(01)00454-3.

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Title Copper in disorders with neurological symptoms: Alzheimer`s, Menkes, and Wilson Diseases
Author(s) Strausak, Daniel
Mercer, Julian
Dieter, Hermann H.
Stremmel, Wolfgang
Multhaup, Gerd
Journal name Brain research bulletin
Volume number 55
Issue number 2
Start page 175
End page 185
Publisher Elsevier Science
Place of publication New York, NY
Publication date 2001-05-15
ISSN 0361-9230
1873-2747
Keyword(s) Alzheimer’s disease
Menkes
Wilson
copper
neurodegeneration
Summary Copper is an essential element for the activity of a number of physiologically important enzymes. Enzyme-related malfunctions may contribute to severe neurological symptoms and neurological diseases: copper is a component of cytochrome c oxidase, which catalyzes the reduction of oxygen to water, the essential step in cellular respiration. Copper is a cofactor of Cu/Zn-superoxide-dismutase which plays a key role in the cellular response to oxidative stress by scavenging reactive oxygen species. Furthermore, copper is a constituent of dopamine-β-hydroxylase, a critical enzyme in the catecholamine biosynthetic pathway. A detailed exploration of the biological importance and functional properties of proteins associated with neurological symptoms will have an important impact on understanding disease mechanisms and may accelerate development and testing of new therapeutic approaches. Copper binding proteins play important roles in the establishment and maintenance of metal-ion homeostasis, in deficiency disorders with neurological symptoms (Menkes disease, Wilson disease) and in neurodegenerative diseases (Alzheimer’s disease). The Menkes and Wilson proteins have been characterized as copper transporters and the amyloid precursor protein (APP) of Alzheimer’s disease has been proposed to work as a Cu(II) and/or Zn(II) transporter. Experimental, clinical and epidemiological observations in neurodegenerative disorders like Alzheimer’s disease and in the genetically inherited copper-dependent disorders Menkes and Wilson disease are summarized. This could provide a rationale for a link between severely dysregulated metal-ion homeostasis and the selective neuronal pathology.
Language eng
DOI 10.1016/S0361-9230(01)00454-3
Field of Research 060410 Neurogenetics
HERDC Research category C1 Refereed article in a scholarly journal
Copyright notice ©2001 Elsevier Science Inc.
Persistent URL http://hdl.handle.net/10536/DRO/DU:30001373

Document type: Journal Article
Collection: School of Biological and Chemical Sciences
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