| Project/Area Number |
25860733
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Multi-year Fund |
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| Research Field |
Neurology
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| Research Institution | National Center of Neurology and Psychiatry |
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Principal Investigator |
UEYAMA Morio 独立行政法人国立精神・神経医療研究センター, 神経研究所 疾病研究第四部, 科研費研究員 (20386593)
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| Project Period (FY) |
2013-04-01 – 2015-03-31
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| Project Status |
Completed (Fiscal Year 2014)
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| Budget Amount *help |
¥4,160,000 (Direct Cost: ¥3,200,000、Indirect Cost: ¥960,000)
Fiscal Year 2014: ¥1,820,000 (Direct Cost: ¥1,400,000、Indirect Cost: ¥420,000)
Fiscal Year 2013: ¥2,340,000 (Direct Cost: ¥1,800,000、Indirect Cost: ¥540,000)
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| Keywords | 脳・神経 / 脳神経疾患 / RNA / 遺伝子 / ショウジョウバエ / RNA結合蛋白質 |
|---|
| Outline of Final Research Achievements |
Amyotrophic lateral sclerosis (ALS) is a devastating disease with movement disorder characterized by degeneration and loss of motor neurons. Recently, an abnormal expansion of GGGGCC repeat in the untranslated region of C9ORF72 gene has been found to be responsible for familial ALS. However, its molecular mechanism leading to ALS pathogenesis remains unclear. To elucidate the pathogenic mechanisms of ALS caused by an expanded repeat, we had established a novel Drosophila model of ALS expressing the GGGGCC repeat RNA. Next, we found that one of the causative gene of ALS, FUS genetically interacts with GGGGCC repeat, indicating that FUS contributes to pathogenic mechanisms of ALS related to GGGGCC repeat expansion.
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