First Author | Kodama H | Year | 1991 |
Journal | J Inherit Metab Dis | Volume | 14 |
Issue | 6 | Pages | 896-901 |
PubMed ID | 1779648 | Mgi Jnum | J:617 |
Mgi Id | MGI:49153 | Doi | 10.1007/BF01800470 |
Citation | Kodama H, et al. (1991) Genetic expression of Menkes disease in cultured astrocytes of the macular mouse. J Inherit Metab Dis 14(6):896-901 |
abstractText | 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. |