Elucidation of pathogenic mechanisms of myasthenia gravis caused by MuSK antibodies using a new synchronized experimental animal model
Project/Area Number |
21591102
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Neurology
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Research Institution | Tokyo Metropolitan Geriatric Hospital and Institute of Gerontology |
Principal Investigator |
SHIGEMOTO Kazuhiro 地方独立行政法人東京都健康長寿医療センター(東京都健康長寿医療センター研究所), 東京都健康長寿医療センター研究所, 研究部長 (40284400)
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Co-Investigator(Renkei-kenkyūsha) |
KONISHI Tetsuro 独立行政法人国立病院機構宇多野病院, 神経内科, 院長 (50426508)
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Project Period (FY) |
2009 – 2011
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Project Status |
Completed (Fiscal Year 2011)
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Budget Amount *help |
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2011: ¥130,000 (Direct Cost: ¥100,000、Indirect Cost: ¥30,000)
Fiscal Year 2010: ¥2,080,000 (Direct Cost: ¥1,600,000、Indirect Cost: ¥480,000)
Fiscal Year 2009: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
|
Keywords | 重症筋無力症 / 神経筋接合部 / MuSK / 動物疾患モデル / 治療薬 / 疾患モデル / muscle-specific kians / 重病筋無力症 |
Research Abstract |
We generated a new animal model in which 100% of mice synchronously develop experimental animal model of myasthenia gravis after immunization with MuSK protein. Our model provides a valuable platform from which to evaluate the role of MuSK signaling in NMJ maintenance and the immune mechanisms and pathophysiology of MuSK-MG, and we propose that 3, 4-DAP may be effective as a symptomatic therapy.
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Report
(4 results)
Research Products
(47 results)
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[Journal Article] 3,4-Diaminopyridine improves neuromuscular transmission in a MuSK antibody-induced mouse model of myasthenia gravis2012
Author(s)
Mori, S. , Kishi, M., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T., Konishi, T., Maruyama, N., and Shigemoto, K.
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Journal Title
Journal of neuroimmunology
Volume: 245
Issue: 1-2
Pages: 75-78
DOI
Related Report
Peer Reviewed
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[Journal Article] Antibodies against muscle-specific kinase impair both presynaptic and postsynaptic functions in a murine model of myasthenia gravis2012
Author(s)
Mori S, Kubo S, Akiyoshi T, Yamada S, Miyazaki T, Hotta H, Desaki J, Kishi M, Konishi T, Nishino Y, Miyazawa A, Maruyama N, Shigemoto K
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Journal Title
The American Journal of Pathology
Volume: 180
Issue: 2
Pages: 617-28
DOI
Related Report
Peer Reviewed
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[Journal Article] Divalent and monovalent autoantibodies cause dysfunction of MuSK by distinct mechanisms in a rabbit model of myasthenia gravis2012
Author(s)
Mori, S. , Yamada, S., Kubo, S. , Chen, J., Matsuda, S. , Shudou, M. , Maruyama, N., and Shigemoto, K.
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Journal Title
Journal of neuroimmunology
Volume: 244
Issue: 1-2
Pages: 1-7
DOI
Related Report
Peer Reviewed
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[Journal Article] Establishment and characterization of hepatocytes from an Immortomouse/SMP30/GNL knockout mouse hybrid lacking vitamin C to study vitamin C transport2011
Author(s)
Amano, A., Handa, S., Aigaki, T., Shigemoto, K., Maruyama, N., Ishigami, A.
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Journal Title
Journal of biochemistry
Volume: 150
Issue: 6
Pages: 671-678
DOI
Related Report
Peer Reviewed
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[Journal Article] Muscle weakness and neuromuscular junctions in aging and disease2010
Author(s)
Shigemoto, K., Kubo, S., Mori, S., Yamada, S., Akiyoshi, T., and Miyazaki, T.
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Journal Title
Geriatr Gerontol Int 10 Suppl
Volume: 1
Issue: s1
Pages: 137-147
DOI
NAID
Related Report
Peer Reviewed
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[Journal Article] Muscle weakness and neuromuscular junction in aging and disease.2010
Author(s)
Shigemoto, K., Kubo, S., Mori, S., Yamada, S., Akiyoshi, T., Miyazaki, T.
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Journal Title
Geriatr.Gerintol.Int.
Volume: 10
NAID
Related Report
Peer Reviewed
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[Journal Article] The role of zinc finger protein 521/early hematopoietic zincfinger protein in erythroid cell differentiation2009
Author(s)
Matsubara, E., Sakai, I., Yamanouchi, J., Fujiwara, H., Yakushijin, Y., Hato, T., Shigemoto, K., Yasukawa, M.
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Journal Title
J Biol Chem 284
Pages: 3480-3487
Related Report
Peer Reviewed
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[Presentation] New strategy to treatment of myasthenia gravis caused by MUSK antibodies using a synchronized experimental animal model2010
Author(s)
Mori, S., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T., Shigemoto, K.
Organizer
第33回日本神経科学会大会
Place of Presentation
神戸
Related Report
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[Presentation] Complement activation is dispensable for myasthenia gravis caused by MUSK antibodies.2010
Author(s)
Shigemoto, K, Mori, S., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T
Organizer
10th International Congress of Neuroimmunology
Place of Presentation
Barcelona
Related Report
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[Presentation] Strategy to treatment of myasthenia gravis caused by MUSK antibodies using a synchronized experimental animal model.2010
Author(s)
Mori, S., Kubo, S., Akiyoshi, T., Yamada, S., Miyazaki, T., Shieemoto, K
Organizer
Society for Neuroscience 2010
Place of Presentation
San Diego
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[Book] Myasthenia gravis Disease Mechanisms and Immune Intervention2009
Author(s)
Shigemoto, K., Kubo, S., Mori.S., Yamada, S., Miyazaki, T., Akiyoshi, T, Maruyama, S.
Total Pages
20
Publisher
Linus Publication, Inc.
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