Spinocerebellar ataxia and distrubance in lipid metabolism
| Project/Area Number |
16K08276
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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 |
Pharmacology in pharmacy
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| Research Institution | Kumamoto University |
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Principal Investigator |
Seki Takahiro 熊本大学, 大学院生命科学研究部(薬), 准教授 (50335650)
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| Project Period (FY) |
2016-04-01 – 2019-03-31
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| Project Status |
Completed (Fiscal Year 2018)
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| Budget Amount *help |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2018: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2017: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2016: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 脊髄小脳失調症 / 脂肪滴 / リピドミクス解析 / 脂質代謝 |
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| Outline of Final Research Achievements |
To elucidate the common pathogenic mechanism of spinocerebellar ataxia, which is caused by various causal genes, we analyzed lipid droplets (LDs), which are organelles containing neutral lipids. When we expressed the causal proteins of SCA21, SCA34, SCA38 and SCA3 in HeLa cells, LDs were significantly decreased compared with the cells expressing wild-type proteins. On the other hand, LDs were significantly increased by the expression of SCA14-causing protein. These findings strongly suggest that disturbance of intracellular lipid metabolism would be a common molecular basis in the pathogenesis of SCA.
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| Academic Significance and Societal Importance of the Research Achievements |
SCAは原因遺伝子座の違いによりSCA1-48に分類されており、様々な原因遺伝子が同定されているが、その遺伝子がコードするタンパク質の機能は様々である。しかし、SCAでは小脳の萎縮と運動失調という共通の症状が観察されるため、発症には共通の分子機序が存在すると想定されるが、現在までにその共通の分子機序は分かっていない。本研究はその共通の分子機序の一つとして脂質代謝異常を提唱するものであり、学術的意義は大きい。また、脂質代謝異常がSCAに共通の治療薬・予防薬の開発に向けた新たな治療標的となることが期待され、社会的意義も大きい。
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Report
(4 results)
Research Products
(92 results)
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[Journal Article] Pharmacological induction of heat shock proteins ameliorates toxicity of mutant PKCγ in spinocerebellar ataxia type 142018
Author(s)
Nakazono, A., Adachi, N., Takahashi, H., Seki, T., Hamada, D., Ueyama, T., Sakai, N. and Saito, N.
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Journal Title
J. Biol. Chem.
Volume: 293
Issue: 38
Pages: 14758-14774
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] Inhibition of Leukotriene B4 Action Mitigates Intracerebral Hemorrhage-Associated Pathological Events in Mice2017
Author(s)
Hijioka, M., Anan, J., Ishibashi, H., Kurauchi, Y., Hisatsune, A., Seki, T., Koga, T., Yokomizo, T., Shimizu, T.,Katsuki, H.
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Journal Title
J Pharmacol Exp Ther
Volume: 360
Issue: 3
Pages: 399-408
DOI
Related Report
Peer Reviewed
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