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Mitochondrial dysfunction in neurodegenerative diseases associated with copper imbalance.
Neurochem Res 2004; 29(3):493-504NR

Abstract

Copper is an essential transition metal ion for the function of key metabolic enzymes, but its uncontrolled redox reactivity is source of reactive oxygen species. Therefore a network of transporters strictly controls the trafficking of copper in living systems. Deficit, excess, or aberrant coordination of copper are conditions that may be detrimental, especially for neuronal cells, which are particularly sensitive to oxidative stress. Indeed, the genetic disturbances of copper homeostasis, Menkes' and Wilson's diseases, are associated with neurodegeneration. Furthermore, copper interacts with the proteins that are the hallmarks of neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease, prion diseases, and familial amyotrophic lateral sclerosis. In all cases, copper-mediated oxidative stress is linked to mitochondrial dysfunction, which is a common feature of neurodegeneration. In particular we recently demonstrated that in copper deficiency, mitochondrial function is impaired due to decreased activity of cytochrome c oxidase, leading to production of reactive oxygen species, which in turn triggers mitochondria-mediated apoptotic neurodegeneration.

Authors+Show Affiliations

Department of Biology, "Tor Vergata" University of Rome, Via della Ricerca Scientifica, 00133 Rome, Italy.No affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

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

Language

eng

PubMed ID

15038597

Citation

Rossi, Luisa, et al. "Mitochondrial Dysfunction in Neurodegenerative Diseases Associated With Copper Imbalance." Neurochemical Research, vol. 29, no. 3, 2004, pp. 493-504.
Rossi L, Lombardo MF, Ciriolo MR, et al. Mitochondrial dysfunction in neurodegenerative diseases associated with copper imbalance. Neurochem Res. 2004;29(3):493-504.
Rossi, L., Lombardo, M. F., Ciriolo, M. R., & Rotilio, G. (2004). Mitochondrial dysfunction in neurodegenerative diseases associated with copper imbalance. Neurochemical Research, 29(3), pp. 493-504.
Rossi L, et al. Mitochondrial Dysfunction in Neurodegenerative Diseases Associated With Copper Imbalance. Neurochem Res. 2004;29(3):493-504. PubMed PMID: 15038597.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Mitochondrial dysfunction in neurodegenerative diseases associated with copper imbalance. AU - Rossi,Luisa, AU - Lombardo,Marco F, AU - Ciriolo,Maria R, AU - Rotilio,Giuseppe, PY - 2004/3/25/pubmed PY - 2004/6/30/medline PY - 2004/3/25/entrez SP - 493 EP - 504 JF - Neurochemical research JO - Neurochem. Res. VL - 29 IS - 3 N2 - Copper is an essential transition metal ion for the function of key metabolic enzymes, but its uncontrolled redox reactivity is source of reactive oxygen species. Therefore a network of transporters strictly controls the trafficking of copper in living systems. Deficit, excess, or aberrant coordination of copper are conditions that may be detrimental, especially for neuronal cells, which are particularly sensitive to oxidative stress. Indeed, the genetic disturbances of copper homeostasis, Menkes' and Wilson's diseases, are associated with neurodegeneration. Furthermore, copper interacts with the proteins that are the hallmarks of neurodegenerative disorders, such as Alzheimer's disease, Parkinson's disease, prion diseases, and familial amyotrophic lateral sclerosis. In all cases, copper-mediated oxidative stress is linked to mitochondrial dysfunction, which is a common feature of neurodegeneration. In particular we recently demonstrated that in copper deficiency, mitochondrial function is impaired due to decreased activity of cytochrome c oxidase, leading to production of reactive oxygen species, which in turn triggers mitochondria-mediated apoptotic neurodegeneration. SN - 0364-3190 UR - https://www.unboundmedicine.com/medline/citation/15038597/Mitochondrial_dysfunction_in_neurodegenerative_diseases_associated_with_copper_imbalance_ L2 - http://ovidsp.ovid.com/ovidweb.cgi?T=JS&PAGE=linkout&SEARCH=15038597.ui DB - PRIME DP - Unbound Medicine ER -