Analyses of regulatory mechanisms of TDP-43 level and risk factor of ALS
Project/Area Number |
24300122
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Partial Multi-year Fund |
Section | 一般 |
Research Field |
Neuroscience in general
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Research Institution | Jikei University School of Medicine |
Principal Investigator |
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Research Collaborator |
HARA Chikako 東京慈恵会医科大学, 医学部, 助教 (40528452)
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Project Period (FY) |
2012-04-01 – 2015-03-31
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Project Status |
Completed (Fiscal Year 2014)
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Budget Amount *help |
¥18,850,000 (Direct Cost: ¥14,500,000、Indirect Cost: ¥4,350,000)
Fiscal Year 2014: ¥6,110,000 (Direct Cost: ¥4,700,000、Indirect Cost: ¥1,410,000)
Fiscal Year 2013: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000)
Fiscal Year 2012: ¥6,760,000 (Direct Cost: ¥5,200,000、Indirect Cost: ¥1,560,000)
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Keywords | ALS / TDP-43 / RNA結合タンパク質 |
Outline of Final Research Achievements |
TDP-43 gene has been identified as a causative gene of amyotrophic lateral sclerosis (ALS) . Two types of mutant TDP-43 knock-in (mTDP-43 KI) mice with mutations in different sites of the gene were generated to investigate the biological effects of each mutation. Considerable phenotypes were observed in mTDP-43 KI mice: poor weight gain and loss of spinal motor neurons, which is related to ALS symptoms. By Exon Array and RT-PCR, defects of RNA metabolism following TDP-43 dysfunction were detected in the mononuclear cells from peripheral blood of KI mice. Furthermore, up-regulation of mutant TDP-43 was detected in astrocytes adjacent to motor neurons from KI mice. Our results, referencing TDP-43 mutations, will provide new insights into the pathophysiology of ALS.
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Report
(4 results)
Research Products
(16 results)
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[Journal Article] The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis2013
Author(s)
Nishimoto, Y., Nakagawa, S., Hirose, T., Okano, H. J., Takao, M., Shibata, S., Suyama, S., Kuwako, K., Imai, T., Murayama, S., Suzuki, N., and Okano, H
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Journal Title
Mol. Brain
Volume: 6
Issue: 1
Pages: 31-31
DOI
Related Report
Peer Reviewed
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[Presentation] Multimodal and exclusive pathology between ALS and FTLD caused by TDP-43 mutations2014
Author(s)
Hara-Miyauchi C, Date Y, Hasegawa M, Kobayashi R, Fujigasaki J, Kogo N, Sano C, Kobayashi Y, Suzuki N, Itohara S, Okano H, Okano HJ
Organizer
米国神経科学会
Place of Presentation
Walter E Washington Convention Center, Washington D. C. U.S.A.
Year and Date
2014-11-15 – 2014-11-19
Related Report
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