| Project/Area Number |
17K08488
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
General anatomy (including histology/embryology)
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| Research Institution | Niigata College of Nursing (2019-2020)
Kagoshima University (2017-2018) |
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Principal Investigator |
Horie Masao 新潟県立看護大学, 看護学部, 教授 (70322716)
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| Project Period (FY) |
2017-04-01 – 2021-03-31
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| Project Status |
Completed (Fiscal Year 2020)
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| Budget Amount *help |
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2019: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2018: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2017: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
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| Keywords | ジストニン / マウス / ジストニア / グリア細胞 / Dystonin / ataxia / 末梢神経 / マクロファージ / 神経系 |
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| Outline of Final Research Achievements |
The Dystonin gene (Dst) is responsible for dystonia musculorum (dt), an inherited mouse model of hereditary neuropathy accompanied by progressive motor symptoms such as dystonia. We examined Dst-deficient mice that showed motor abnormalities in homozygous dt23Rbrc/dt23- Rbrc mice are not as severe as homozygous DstGt/DstGt mice.Histological analyses showed abnormal neurofilament(NF) accumulation in the nervous system of homozygous dt23Rbrc/dt23Rbrc mice, which is characteristic of the dt phenotype. We mapped the distribution of abnormal NF-accumulated neurons in the brain and found that theywere located specifically in the brainstem, spinal cord, and in regions such as the vestibular nucleus, reticular nucleus, and red nucleus, which are implicated in posture and motor coordination pathways. which causes histological abnormalities in the central nervous system that may account for the abnormal motor phenotype.
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| Academic Significance and Societal Importance of the Research Achievements |
本研究の結果は、（１）シュワン細胞におけるはDstタンパクの不活性化はジストニア様症状の直接的な原因ではないが、歩様異常の原因となること、（２）全身性ジストニア様症状発症には、シュワン細胞でのDstタンパクの不活性化に加えて、他の末梢神経あるいは中枢神経においてもDstタンパクの不活性化が必要であることが示唆している。これらの知見は、今後の神経難病であるジストニアの治療法の開発研究に一歩になる。
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