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Int J Biochem Cell Biol. 2018 Sep;102:71-75. doi: 10.1016/j.biocel.2018.07.001. Epub 2018 Jul 8.

Mitochondrial copper homeostasis and its derailment in Wilson disease.

Author information

1
Institute of Molecular Toxicology and Pharmacology, Helmholtz Center Munich, German Research Center for Environmental Health, 85764 Neuherberg, Germany; Institute of Toxicology and Environmental Hygiene, Technical University of Munich, 80802 Munich, Germany. Electronic address: zischka@helmholtz-muenchen.de.
2
Institute of Molecular Toxicology and Pharmacology, Helmholtz Center Munich, German Research Center for Environmental Health, 85764 Neuherberg, Germany.

Abstract

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.

KEYWORDS:

Copper; Liver; Mitochondria; Wilson disease

PMID:
29997057
DOI:
10.1016/j.biocel.2018.07.001
[Indexed for MEDLINE]
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