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D Huster - Annals of the New York Academy of Sciences, 2014 - Wiley Online Library

Structural and metabolic changes in Atp7b−/− mouse liver and potential for new interventions in Wilson's disease.

Stem education interventions

[No authors listed]

GSID: BAnDim5PXwcJ

Excerpt

Wilson's disease (WD) is caused by ATP7B mutations and results in copper accumulation and toxicity in liver and brain tissues. The specific mechanisms underlying copper toxicity are still poorly understood. Mouse models have revealed new insights into pathomechanisms of hepatic WD. Mitochondrial damage is observed in livers of WD patients and in mouse models; copper induces fragmentation of mitochondrial membrane lipids, particularly cardiolipin, with deleterious effects on both mitochondrial integrity and function …

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