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Kodama H, Meguro Y, Abe T, et al. Genetic expression of Menkes disease in cultured astrocytes of the macular mouse. J Inherit Metab Dis. 1991;14(6):896-901doi: 10.1007/BF01800470.
Kodama, H., Meguro, Y., Abe, T., Rayner, M. H., Suzuki, K. T., Kobayashi, S., & Nishimura, M. (1991). Genetic expression of Menkes disease in cultured astrocytes of the macular mouse. Journal of inherited metabolic disease, 14(6), 896-901. https://doi.org/10.1007/BF01800470
Kodama, H, et al. "Genetic expression of Menkes disease in cultured astrocytes of the macular mouse." Journal of inherited metabolic disease vol. 14,6 (1991): 896-901. doi: https://doi.org/10.1007/BF01800470
Kodama H, Meguro Y, Abe T, Rayner MH, Suzuki KT, Kobayashi S, Nishimura M. Genetic expression of Menkes disease in cultured astrocytes of the macular mouse. J Inherit Metab Dis. 1991;14(6):896-901. doi: 10.1007/BF01800470. PMID: 1779648.
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J Inherit Metab Dis. 1991;14(6):896-901. doi: 10.1007/BF01800470.

Genetic expression of Menkes disease in cultured astrocytes of the macular mouse.

Journal of inherited metabolic disease

H Kodama, Y Meguro, T Abe, M H Rayner, K T Suzuki, S Kobayashi, M Nishimura

Affiliations

  1. Department of Pediatrics, Teikyo University, School of Medicine, Tokyo, Japan.

PMID: 1779648 DOI: 10.1007/BF01800470

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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