Mechanism of motor neuron death in ALS: transcription activity of ribosomal RNA gene and TDP-43
| Project/Area Number |
22300117
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Nerve anatomy/Neuropathology
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| Research Institution | Shinshu University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
MIZUTANI Toshio 公益財団法人東京都医学総合研究所, 東京都神経科学総合研究所, 研究員 (40124416)
YAMAZAKI Mineo 日本医科大学, 医学部, 准教授 (10277577)
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| Project Period (FY) |
2010 – 2012
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| Project Status |
Completed (Fiscal Year 2012)
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| Budget Amount *help |
¥16,900,000 (Direct Cost: ¥13,000,000、Indirect Cost: ¥3,900,000)
Fiscal Year 2012: ¥3,380,000 (Direct Cost: ¥2,600,000、Indirect Cost: ¥780,000)
Fiscal Year 2011: ¥3,900,000 (Direct Cost: ¥3,000,000、Indirect Cost: ¥900,000)
Fiscal Year 2010: ¥9,620,000 (Direct Cost: ¥7,400,000、Indirect Cost: ¥2,220,000)
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| Keywords | 神経変性疾患 / ALS / RNA / ストレス顆粒 / TDP-43 / リボゾームRNA遺伝子転写活性 / Bunina小体 / FUS |
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| Research Abstract |
The present study elucidate the abnormalities in the protein synthesis system in the motor neurons in the spinal cords in patients with amyotrophic lateral sclerosis (ALS). Those abnormalities are as follows: 1) decrease of ribosomal (r) RNA gene transcription activity, 2) correlation between rRNA gene activity and amount of nucleolin in the nucleus, 3) aggregation of the molecular chaperone, and 4) seeding each other in aggregation of molecular chaperone and cystatin C, which is a component of the Bunina body.
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Report
(4 results)
Research Products
(52 results)
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[Journal Article] Familial ALS with FUS P525L mutation: two Japanese sisters with multiple system involvement.2012
Author(s)
Mochizuki Y, Isozaki E, Takao M, Hashimoto T, Shibuya M, Arai M, Hosokawa M, Kawata A, Oyanagi K, Mihara B, Mizutani T
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Journal Title
Journal of the Neurological Sciences
Volume: 323
Issue: 1-2
Pages: 85-92
DOI
Related Report
Peer Reviewed
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[Journal Article] A Case of Dural Arteriovenous Fistulas at the Craniocervical Junction Presenting with Occipital/Neck Pain Associated with Sleep2012
Author(s)
Suda, S., Katsura, K., Okubo, S., Abe, A., Kanamaru, T., Ueda, M., Mishina, M., Yamazaki, M., Katayama, Y.
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Journal Title
Internal Medicine
Volume: 51
Issue: 8
Pages: 925-928
DOI
NAID
ISSN
0918-2918, 1349-7235
Related Report
Peer Reviewed
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[Journal Article] Survival motor neuron (SMN)protein in the spinal anterior horn cells of patients with sporadic amyotrophic lateral sclerosis2011
Author(s)
Piao, Y., Hashimoto, T., Takahama, S., Kakita, A., Komori, T., Morita,T., Takahashi, H., Mizutani, T., Oyanagi, K.
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Journal Title
Brain Research
Volume: 1327
Pages: 152-159
NAID
Related Report
Peer Reviewed
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[Journal Article] Nasu-Hakola disease with a splicing mutation of TREM2 in a Japanese family2011
Author(s)
Numasawa, Y., Yamaura, C., Ishihara, S., Shintani, S., Yamazaki, M., Tabunoki, H., Satoh, JI.
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Journal Title
European Journal of Neurology
Volume: 18
Issue: 9
Pages: 1179-1183
DOI
Related Report
Peer Reviewed
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[Presentation] Distinct clinicopathological features in two large families with familial amyotrophic lateral sclerosis having L106V mutation in the copper/zinc superoxide dismutase (SOD1) gene2011
Author(s)
Hineno, A., Nakamura, A., Shimojima, Y., Yoshida, K., Oyanagai, K., Ikeda, S.
Organizer
第2回アジア神経病理学会
Place of Presentation
北京
Year and Date
2011-11-05
Related Report
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[Book] Parkinsonism-dementia complex2011
Author(s)
Oyanagi K, Hashimoto T, Yamazaki M
Publisher
In: Neurodegeneration: The Molecular Pathology of Dementia and Movement Disorders (Eds: Weller RO, Dickson DW) Springer, Oxford
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