Kodama H, Meguro Y, Abe T, Rayner M H, Suzuki K T, Kobayashi S, Nishimura M
Department of Pediatrics, Teikyo University, School of Medicine, Tokyo, Japan.
J Inherit Metab Dis. 1991;14(6):896-901. doi: 10.1007/BF01800470.
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.
