Analysis of mRNA metabolisms and therapeutic interventions for defects in neuromuscular signal transduction
Project/Area Number |
21390266
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Neurology
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Research Institution | Nagoya University |
Principal Investigator |
OHNO Kinji 名古屋大学, 大学院・医学系研究科, 教授 (80397455)
|
Co-Investigator(Kenkyū-buntansha) |
MASUDA Akio 名古屋大学, 大学院・医学系研究科, 助教 (10343203)
ITO Mikako 名古屋大学, 大学院・医学系研究科, 助教 (60444402)
|
Project Period (FY) |
2009 – 2011
|
Project Status |
Completed (Fiscal Year 2011)
|
Budget Amount *help |
¥17,810,000 (Direct Cost: ¥13,700,000、Indirect Cost: ¥4,110,000)
Fiscal Year 2011: ¥5,720,000 (Direct Cost: ¥4,400,000、Indirect Cost: ¥1,320,000)
Fiscal Year 2010: ¥5,720,000 (Direct Cost: ¥4,400,000、Indirect Cost: ¥1,320,000)
Fiscal Year 2009: ¥6,370,000 (Direct Cost: ¥4,900,000、Indirect Cost: ¥1,470,000)
|
Keywords | 先天性筋無力症候群 / スプライシング異常 / protein-anchoring therapy / 終板アセチルコリンエステラーゼ欠損症 / Collagen Q / アセチルコリン受容体 / スプライシングトランス因子 / hnRNP L / hnRNP LL |
Research Abstract |
We previously reported a splicing mutation in exon P3A of CHRNA1. We here identified that hnRNP L binds to the mutant site and suppresses splicing of exon P3A. We also reported that intravenous and intramuscular administration of AAV-COLQ to Colq-/-mice improved motor functions, as well as electrophysiological, histochemical, and biochemical features.
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Report
(4 results)
Research Products
(43 results)
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[Journal Article] A novel mutation in SCN4A causes severe myotonia and school-age-onset paralytic episodes2012
Author(s)
Yoshinaga H, Sakoda S, Good JM, Takahashi MP, Kubota T, Arikawa-Hirasawa E, Nakata T, OhnoK, Kitamura T, Kobayashi K, Ohtsuka Y
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Journal Title
J Neurol Sci
Volume: 315
Pages: 15-19
Related Report
Peer Reviewed
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[Journal Article] Ancestral origin of the ATTCT repeat expansion in spinocerebellar ataxia type 10 (SCA10)2009
Author(s)
Almeida T, Alonso I, Martins S, Ramos EM, Azevedo L, Ohno K, Amorim A, Saraiva-Pereira ML, Jardim LB, Matsuura T, Sequeiros J, Silveira I.
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Journal Title
Related Report
Peer Reviewed
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[Journal Article] Molecular hydrogen is protective against 6-hydroxydopamine-induced nigrostriatal degeneration in a rat model of Parkinson's disease2009
Author(s)
Fu Y, Ito M, Fujita Y, Ito M, Ichihara M, Masuda A, Suzuki Y, Maesawa S, Kajita Y, Hirayama M, Ohsawa I, Ohta S, Ohno K.
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Journal Title
Neurosci Lett Vol. 453
Pages: 81-85
Related Report
Peer Reviewed
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[Presentation] Anti-MuSK antibodies in myastheniagravis block binding of collagen Q toMuSK expressed at theneuromuscular junction2011
Author(s)
Kawakami Y, Ito M, Hirayama M, Sahashi K, Ohkawara B, Masuda A, Hishida H, Mabuchi N, Engel AG
Organizer
41st Annual Meeting, Society for Neuroscience
Place of Presentation
Washington DC, USA
Year and Date
2011-11-15
Related Report
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[Presentation] Anti-MuSK antibodies in myasthenia gravis block binding of collagen Q to MuSK expressed at the neuromuscular junction2011
Author(s)
Kawakami Y, Ito M, Hirayama M, Sahashi K, Ohkawara B, Masuda A, Hishida H, Mabuchi N, Engel AG, Ohno K
Organizer
41st Annual Meeting, Society for Neuroscience
Place of Presentation
Washington DC, USA
Year and Date
2011-11-15
Related Report
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