| Project/Area Number |
02670436
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| Research Category |
Grant-in-Aid for General Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pediatrics
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| Research Institution | Shiga University of Medical Science |
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Principal Investigator |
YAMANO Tsunekazu Shiga University of Medical Science Pediatrics, Asociated Professor., 医学部, 助教授 (20093172)
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| Project Period (FY) |
1990 – 1991
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| Project Status |
Completed (Fiscal Year 1991)
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| Budget Amount *help |
¥2,000,000 (Direct Cost: ¥2,000,000)
Fiscal Year 1991: ¥900,000 (Direct Cost: ¥900,000)
Fiscal Year 1990: ¥1,100,000 (Direct Cost: ¥1,100,000)
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| Keywords | Menkes kinky Hair Disease / Macular Mouse / Coppoer Metabolis / Brain Tissue Transplantation / Mitochondrial Abnormality / macular マウス / 銅代謝異常 |
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| Research Abstract |
The macular mutant mouse shows X-linked recessive inheritance. Its hemizygote with white fur color and curly whiskers manifests tonic seizures and ataxia around day 10 and dies around day 15 with severe emaciation. In its brain, copper content is decreased and cytochrome c oxidase (CCO) activity is also reduced. Additionally arborization of neurons is retarded and abnormal mitochoindria are observed in them. Thus, the hemizgote is considered as a model of Menkes kinky hair disease (MKHD). The study was undertaken to elucidate the pathogenesis of MKHD using this model mouse.
Tissues from cerebral cortex of 18-day old embryos of hemizy-gote and normal littermate were transplanted into cerebral cortex of 2-day-old normal littermates and heterozygotes, respectively. Each grafts were examined on day 10 and 14 of age by the Golgi stain, histochemical stain for CCO activity, and electron microscopy.
Neurons in graft of hemizygote did not show catch-up in the arborization and CCO activity. Abnormal mitochondria were also seen in them 12 days after transplantation. On the other hand, Neurons in graft of normal littermate grown in the cerebrum of the hemizygote did not show pathological changes.
These results suggest that neuronal degeneration dose not only depend on copper insufficiency in the hemizygote.
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