Analysis of ALS related gene products in the mouse central nerve system
| Project/Area Number |
26450403
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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 |
Veterinary medical science
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| Research Institution | Shiga University of Medical Science |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
URUSHITANI Makoto 滋賀医科大学, 医学部, 教授 (60332326)
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| Project Period (FY) |
2014-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2016: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2015: ¥1,040,000 (Direct Cost: ¥800,000、Indirect Cost: ¥240,000)
Fiscal Year 2014: ¥2,990,000 (Direct Cost: ¥2,300,000、Indirect Cost: ¥690,000)
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| Keywords | 筋萎縮性側索硬化症(ALS) / 小胞体ストレス / 子宮内エレクトロポレーション / 孤発性ALS / オートファジー / in utero electroporation / ALS / ユビキチン / 筋萎縮性側索硬化症（ALS） |
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| Outline of Final Research Achievements |
A nuclear protein, TDP-43, accumulates in the cytoplasmic inclusions in the sporadic ALS lesions with post-transcriptional modifications, such as ubuiquitination, phosphorylation and truncation, and it works as a pathologic protein of the disease. Motor neurons, target cells of ALS, are highly sensitive to stress in the ER, which is the specialized organelle for folding and maturation of membrane and secretory proteins. I observed that the integrated responses against ER stress is increased in the HeLa cells lacking TDP-43. Comprehensive analysis revealed that a protein, which may regulate ER homeostasis, is drastically down-regulated in the TDP-43-deficient cells. In addition, I performed immune histological examination using the cerebral cortices which was transfected with the TDP-43 regulated gene by in utero electroporation.
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Report
(4 results)
Research Products
(8 results)
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[Journal Article] CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.2016
Author(s)
Uchida T, Tamaki Y, Ayaki T, Shodai A, Kaji S, Morimura T, Banno Y, Nishitsuji K, Sakashita N, Maki T, Yamashita H, Ito H, Takahashi R, Urushitani M.
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Journal Title
Scientific Reports
Volume: 6
Issue: 1
Pages: 19118-19136
DOI
NAID
Related Report
Peer Reviewed / Open Access
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