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Zischka, H. ; Borchard, S.

Mitochondrial copper toxicity with a focus on Wilson disease.

In: Clinical and Translational Perspectives on WILSON DISEASE. 2019. 64-75
DOI
In Wilson disease (WD), toxic copper overload occurs due to an impaired copper excretion from the liver. Mitochondria, the power plants of all higher eukaryotic cells, are fundamentally dependent on copper to fulfill their bioenergetic tasks. This is ensured by the regulated routing of copper to these organelles. However, in WD patients and animal models, mitochondria are directly and progressively damaged by copper overload. This leads to increasing, characteristic mitochondrial structure alterations and to severe bioenergetic impairments. Finally, mitochondrial destruction is paralleled by liver failure and death. Especially, interventions with copper-chelators that cause efficient reduction of the mitochondrial copper load result in the restoration of their structure and function, avoid acute liver failure in WD and are thus promising options for future WD therapies.
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Publikationstyp Artikel: Sammelbandbeitrag/Buchkapitel
Schlagwörter Atp7b ; Copper ; Lpp Rat ; Mitochondria ; Reactive Oxygen Species (ros) ; Tx Mouse ; Tx-j Mouse ; Wilson Disease
Sprache englisch
Veröffentlichungsjahr 2019
Prepublished im Jahr 2018
HGF-Berichtsjahr 2018
Bandtitel Clinical and Translational Perspectives on WILSON DISEASE
Quellenangaben Band: , Heft: , Seiten: 64-75 Artikelnummer: , Supplement: ,
Institut(e) Institute of Molecular Toxicology and Pharmacology (TOX)
POF Topic(s) 30203 - Molecular Targets and Therapies
Forschungsfeld(er) Enabling and Novel Technologies
PSP-Element(e) G-505200-003
DOI 10.1016/B978-0-12-810532-0.00008-2
Scopus ID 85092601493
Erfassungsdatum 2018-11-15
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