Genetic expression of Menkes disease in cultured astrocytes of the macular mouse
Journal of Inherited Metabolic Disease1991Vol. 14(6), pp. 896–901
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Hiroko Kodama, Yuki Meguro, Takanori Abe, Michael Rayner, Kazuo Suzuki, S. Kobayashi, Mikio Nishimura
Abstract
The copper concentration was investigated in the cultured astrocytes from macular mice, an animal model of Menkes disease. An excessive amount of copper was accumulated in the astrocytes as copper-metallothionein. These results show that the underlying genetic defect of the macular mouse is expressed in the astrocytes. A similar situation may exist in Menkes disease and cause a failure of copper transport to neurones.
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