2001 Fiscal Year Final Research Report Summary
Glial participation in neuronal disturbance of Niemann-Pick disease type C
| Project/Area Number |
12670788
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | St. Marianna University School of Medicine |
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Principal Investigator |
SAKIYAMA Takeshi St. Marianna University, School of Medicine Pathology, Associate Professor, 医学部, 助教授 (20130510)
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| Co-Investigator(Kenkyū-buntansha) |
SUZUKI Hiroyoshi Sendai National Hospital, 臨床検査科, 科長
ABE Mitsubumi St. Marianna University, School of Medicine Pathology, Assistant Professor, 医学部, 講師 (30231971)
TADOKORO Mamoru St. Marianna University, School of Medicine Pathology, Professor, 医学部, 教授 (80081644)
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| Project Period (FY) |
2000 – 2001
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| Keywords | Niemann-Pick disease type C / Glia / neural disturbance / Niemann-Pick mouse / BMT / animal model |
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| Research Abstract |
Some neurodegenerative disorders have possible disturbance in not only neuron but also glia including astrocytes. In order to establish an effective therapy for neurodegenerative disorders in the future, pathophysiology and chronological changes occurring in several species including human-being were studied. For this purpose, we used Niemann-.Pick type C (NPC) disease. The interrelationship between neuron and glia was.studied among different species such as human, mice and cat. The difference of phenotype was ascertained in that atrophy of cerebellum cortex and deletion of Purkinje cells occurred in human and mice, but not in cat, which showed lipid accumulation in Purkinje cells. The cerebral cortex of mice showed ubiquitin positive substance in neuron and its surrounding macrophages. This positive substance existed also in the white matter and astrocytes. It may disturb the protective function on neuron, and may result in nenronal disturbance. These findings progressed and showed a more diffuse distribution as mice grew older. However, BMT treated NPC mice at 8 weeks of age showed almost no remntfnt of ubiquitinized accumulation, and macrophages were contiguous to neurons. Neuronal change was minimum by light microscopy, although EM findings showed improvement. After BMT, the number of astrocytes decreased and the accumulation was diminished. After BMT, there was no clinical improvement in this disease. However, the fact that accumulation was processed in the brain may suggest future hope in obtaining an effective therapy for slowly progressive neurodegenerative disorders.
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