Article
Evidence for a critical role of ceruloplasmin oxidase activity in iron metabolism of Wilson disease gene knockout mice.
Journal of gastroenterology and hepatology - 1 Jun 2010
Merle Uta, Tuma Sabine, Herrmann Thomas, Muntean Valer, Volkmann Martin, Gehrke Sven G, Stremmel Wolfgang
Abstract excerpt
BACKGROUND AND AIM: Wilson disease is a genetic disorder associated with copper overload due to mutations within the ATP7B gene. Although copper and iron metabolism are closely linked, the influence of mutations of the ATP7B gene on iron homeostasis is unknown. Therefore, the present study was carried out to elucidate iron metabolism in Atp7b(-/-) mice, an animal model of Wilson disease. METHODS: Hepatic iron...
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