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Publication : Localization of Menkes gene expression in the mouse brain; its association with neurological manifestations in Menkes model mice.

First Author  Iwase T Year  1996
Journal  Acta Neuropathol Volume  91
Issue  5 Pages  482-8
PubMed ID  8740228 Mgi Jnum  J:33681
Mgi Id  MGI:81159 Doi  10.1007/s004010050455
Citation  Iwase T, et al. (1996) Localization of Menkes gene expression in the mouse brain; its association with neurological manifestations in Menkes model mice. Acta Neuropathol (Berl) 91(5):482-8
abstractText  Menkes gene (Mel or MNK, encoding putative copper- transporting ATPase) expression was investigated and compared in normal and macular mutant mouse brain. Northern blot analysis showed a distinct 8.3-kb transcript and no obvious difference in size or extent in normal mice and macular mutants on postnatal days 0, 4, 7, 10 or 13. In situ hybridization revealed that certain specific populations of cells in the brain express Menkes mRNA, and that their localization in normal and mutant mice did not differ and was conserved on days 4, 10 and 13. The most intense hybridization signals were observed in the hippocampal CA1 region and dentate gyrus, the olfactory bulb nuclei, the cerebellar granular cell layer, the choroid plexus and the ependyma, with less intense signals in the hippocampal CA3 region and cerebellar Purkinje cells. In addition, necrotic neuronal cell death was predominantly observed in the CA3 region and the Purkinje cells of macular mice after postnatal day 10. The finding that the regions that had lower expression level of Menkes mRNA corresponded to those showing neuronal necrosis suggests that the Menkes gene may be responsible for the neuronal degeneration in some specific portions of the brain and clinical manifestations in this mutant.
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