| Project/Area Number |
15K18370
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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 |
Nerve anatomy/Neuropathology
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| Research Institution | Tokyo Metropolitan Institute of Medical Science |
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Principal Investigator |
TANAKA Yoshinori 公益財団法人東京都医学総合研究所, 認知症・高次脳機能研究分野, 研究員 (00747933)
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| Co-Investigator(Renkei-kenkyūsha) |
HASEGAWA Masato 公益財団法人東京都医学総合研究所, 認知症高次脳機能研究分野, 分野長 (10251232)
NONAKA Takashi 公益財団法人東京都医学総合研究所, 認知症高次脳機能研究分野, 副参事研究員 (30356258)
KAMETANI Fuyuki 公益財団法人東京都医学総合研究所, 認知症高次脳機能研究分野, 主席研究員 (70186013)
SUZUKI Genjiro 公益財団法人東京都医学総合研究所, 認知症高次脳機能研究分野, 主席研究員 (60466034)
HOSOKAWA Masato 公益財団法人東京都医学総合研究所, 認知症高次脳機能研究分野, 主席研究員 (00435116)
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| Project Period (FY) |
2015-04-01 – 2017-03-31
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| Project Status |
Completed (Fiscal Year 2016)
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| Budget Amount *help |
¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
Fiscal Year 2016: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2015: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
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| Keywords | Profilin1 / TDP-43 / ALS / mutation / aggresome / LC3 / seeding / prion / プロフィリン１ / アグリソーム / 筋萎縮性側索硬化症 / プリオン |
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| Outline of Final Research Achievements |
The nuclear protein TDP-43 accumulates at the cytoplasm of motor neurons, as a pathological form, in the patients of amyotrophic lateral sclerosis (ALS). As the role of TDP-43 in the nucleus is essential, the accumulation of pathological TDP-43 is identified as a cause of ALS. In the present study, we investigated the effect of ALS-linked PFN1 gene mutations, a cause of familial ALS characteristic of TDP-43 accumulation, on the accumulation of TDP-43 using cultured cells. The cells expressing mutant PFN1 harbored PFN1 aggregates, and those sequestered TDP-43. Furthermore, TDP-43 sequestered into PFN1 aggregates was partly converted into the pathological form of TDP-43 that promotes TDP-43 accumulation like a pathological prion.
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