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Article

Liver mitochondrial membrane crosslinking and destruction in a rat model of Wilson disease

2 Mar 2011

Abstract excerpt

Wilson disease (WD) is a rare hereditary condition that is caused by a genetic defect in the copper-transporting ATPase ATP7B that results in hepatic copper accumulation and lethal liver failure. The present study focuses on the structural mitochondrial alterations that precede clinical symptoms in the livers of rats lacking Atp7b, an animal model for WD. Liver mitochondria from these Atp7b–/– rats contained...

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PMID: 21364284
DOI: 10.1172/jci45401

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