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Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases.

Abstract

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-beta-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.

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  • Publisher Full Text
  • Authors+Show Affiliations

    ,

    Center for Cellular and Molecular Biology, School of Biological and Chemical Sciences, Deakin University, Burwood, Victoria, Australia.

    , , ,

    Source

    Brain research bulletin 55:2 2001 May 15 pg 175-85

    MeSH

    Alzheimer Disease
    Animals
    Brain
    Copper
    Enzymes
    Hepatolenticular Degeneration
    Homeostasis
    Humans
    Menkes Kinky Hair Syndrome
    Metalloproteins
    Nerve Degeneration

    Pub Type(s)

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

    Language

    eng

    PubMed ID

    11470313

    Citation

    Strausak, D, et al. "Copper in Disorders With Neurological Symptoms: Alzheimer's, Menkes, and Wilson Diseases." Brain Research Bulletin, vol. 55, no. 2, 2001, pp. 175-85.
    Strausak D, Mercer JF, Dieter HH, et al. Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases. Brain Res Bull. 2001;55(2):175-85.
    Strausak, D., Mercer, J. F., Dieter, H. H., Stremmel, W., & Multhaup, G. (2001). Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases. Brain Research Bulletin, 55(2), pp. 175-85.
    Strausak D, et al. Copper in Disorders With Neurological Symptoms: Alzheimer's, Menkes, and Wilson Diseases. Brain Res Bull. 2001 May 15;55(2):175-85. PubMed PMID: 11470313.
    * Article titles in AMA citation format should be in sentence-case
    TY - JOUR T1 - Copper in disorders with neurological symptoms: Alzheimer's, Menkes, and Wilson diseases. AU - Strausak,D, AU - Mercer,J F, AU - Dieter,H H, AU - Stremmel,W, AU - Multhaup,G, PY - 2001/7/27/pubmed PY - 2001/9/8/medline PY - 2001/7/27/entrez SP - 175 EP - 85 JF - Brain research bulletin JO - Brain Res. Bull. VL - 55 IS - 2 N2 - 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-beta-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. SN - 0361-9230 UR - https://www.unboundmedicine.com/medline/citation/11470313/Copper_in_disorders_with_neurological_symptoms:_Alzheimer's_Menkes_and_Wilson_diseases_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0361-9230(01)00454-3 DB - PRIME DP - Unbound Medicine ER -