Modeling ALS with TDP-43 proteinopathy
| Project/Area Number |
15H06225
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| Research Category |
Grant-in-Aid for Research Activity Start-up
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| Allocation Type | Single-year Grants |
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| Research Field |
Neurology
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| Research Institution | Niigata University |
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Principal Investigator |
Sugai Akihiro 新潟大学, 医歯学総合病院, 特任助教 (70758903)
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| Project Period (FY) |
2015-08-28 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥2,990,000 (Direct Cost: ¥2,300,000、Indirect Cost: ¥690,000)
Fiscal Year 2016: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2015: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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| Keywords | ALS / TDP-43 / アンチセンスオリゴ / 自己制御機構 / マウスモデル / iPS細胞モデル / 選択的スプライシング / RNA代謝 / 病態モデル / 筋萎縮性側索硬化症 / スプライシング / アンチセンス核酸 |
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| Outline of Final Research Achievements |
Accumulation of TDP-43 in the cytoplasm of motor neurons is a pathological hallmark of amyotrophic lateral sclerosis (ALS). TDP-43 regulates its own mRNA expression. We recently elucidated that a redundant transcription followed by alternative splicing of the pre-mRNA is a critical process for the auto-regulation and that TDP-43 mRNA is increased in ALS motor neurons. Based on this finding, we disturbed the alternative spicing and developed a model with increased intrinsic TDP-43 in mice spinal cords and in human iPS-cell derived neurons.
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Report
(3 results)
Research Products
(3 results)
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[Journal Article] Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43.2016
Author(s)
Koyama A, Sugai A, Kato T, Ishihara T, Shiga A, Toyoshima Y, Koyama M, Konno T, Hirokawa S, Yokoseki A, Nishizawa M, Kakita A, Takahashi H, Onodera O.
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Journal Title
Nucleic Acids Res
Volume: 44
Issue: 12
Pages: 5820-5836
DOI
Related Report
Peer Reviewed / Open Access / Acknowledgement Compliant
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