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Mitochondrial copper homeostasis and its derailment in Wilson disease.
Int. J. Biochem. Cell Biol. 102, 71-75 (2018)
Publ. Version/Full Text
Postprint
DOI
PMC
In mitochondria, copper is a Janus-faced trace element. While it is the essential cofactor of the mitochondrial cytochrome c oxidase, a surplus of copper can be highly detrimental to these organelles. On the one hand, mitochondria are strictly dependent on adequate copper supply for proper respiratory function, and the molecular mechanisms for metalation of the cytochrome c oxidase have been largely characterized. On the other hand, copper overload impairs mitochondria and uncertainties exist concerning the molecular mechanisms for mitochondrial metal uptake, storage and release. The latter issue is of fundamental importance in Wilson disease, a genetic disease characterized by dysfunctional copper excretion from the liver. Prime consequences of the progressive copper accumulation in hepatocytes are increasing mitochondrial biophysical and biochemical deficits. Focusing on this two-sided aspect of mitochondrial copper, we review mitochondrial copper homeostasis but also the impact of excessive mitochondrial copper in Wilson disease.
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Publication type
Article: Journal article
Document type
Review
Keywords
Mitochondria ; Liver ; Wilson Disease ; Copper; Cytochrome-oxidase; Superoxide-dismutase; Hepatocellular Mitochondria; Saccharomyces-cerevisiae; Electron-transfer; Rat-liver; Gene; Metabolism; Expression; Overload
ISSN (print) / ISBN
1357-2725
e-ISSN
1878-5875
Quellenangaben
Volume: 102,
Pages: 71-75
Publisher
Elsevier
Publishing Place
The Boulevard, Langford Lane, Kidlington, Oxford Ox5 1gb, England
Non-patent literature
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Reviewing status
Peer reviewed