Mitochondrial copper homeostasis and its derailment in Wilson disease

The International Journal of Biochemistry & Cell Biology
Hans Zischka, Claudia Einer

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.

Citations

Aug 14, 2019·Chinese Medical Journal·Zhi-Bin ZhangMing-Sheng Huai
Apr 2, 2020·International Journal of Molecular Sciences·Barbara WittTanja Schwerdtle
Aug 14, 2020·Metallomics : Integrated Biometal Science·C Derrick QuarlesM Paul Field
Jan 25, 2021·Journal of Trace Elements in Medicine and Biology : Organ of the Society for Minerals and Trace Elements (GMS)·Barbara WittTanja Schwerdtle
Jul 25, 2021·International Journal of Molecular Sciences·Xuan WangJunjie Luo
Aug 28, 2021·Biomolecules·Valentina MediciCecilia Giulivi
Aug 31, 2021·The EMBO Journal·Daniel J KlionskyFederico Pietrocola
Jan 14, 2022·Comparative Biochemistry and Physiology. Toxicology & Pharmacology : CBP·Michael O IseiCollins Kamunde

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