Study on the development of treatments for collagen VI related myopathy.
Project/Area Number |
21591094
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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 | Kagoshima University |
Principal Investigator |
HIGUCHI Itsuro 鹿児島大学, 医学部・歯学部附属病院, 講師 (80183573)
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Co-Investigator(Kenkyū-buntansha) |
TAKASHIMA Hiroshi 鹿児島大学, 大学院・医歯学総合研究科, 教授 (80372803)
ARIMURA Kimiyoshi 鹿児島大学, 大学院・医歯学総合研究科, 准教授 (30140908)
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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 |
¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2011: ¥1,170,000 (Direct Cost: ¥900,000、Indirect Cost: ¥270,000)
Fiscal Year 2010: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2009: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
|
Keywords | 6型コラーゲン / Ullrich病 / Bethlemミオパチー / 関節拘縮 / thrombomodulin / 実験的治療 / 細胞接着 / ウルリッヒ病 / ベスレムミオパチー / collagen VI / Ullrich型先天性筋ジストロティー / 皮膚線維芽細胞 / コラーゲンVI |
Research Abstract |
We have been continuing a long-term follow-up of collagen VI related diseases as Ullrich disease and Bethlem myopathy. The main cause of difficulty in walking in Bethlem myopathy was joint contracture, and the ambulatory period was extended by the orthopedic treatment. We have been performing genetic analysis of genes of collagen VI and related proteins. We found that recombinant thrombomodulin can improve the adhesive capacity of fibroblasts and increase the expression of collagen VI in Ullrich disease.
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Report
(4 results)
Research Products
(15 results)
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[Journal Article] A new phenotype of mitochondrial disease characterized by familial late-onset predominant axial myopathy and encephalopathy2011
Author(s)
Sakiyama Y, Okamoto Y, Higuchi I, Inamori Y, Sangatsuda Y, Michizono K, Watanabe O, Hatakeyama H, Goto Y, Arimura K, Takashima H.
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Journal Title
Acta Neuropathol
Volume: 121(6)
Pages: 775-783
Related Report
Peer Reviewed
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[Journal Article] Role of Kenae/CCDC125 in cell motility through the deregulation of RhoGTPase2009
Author(s)
Araya N, Arimura H, Kawahara K, Yagishita N, Ishida J, Fujii R, Aratani S, Fujita H, Sato T, Yamano Y, Higuchi I, Osame M, Nishioka K, Fukamizu A, Arimura K, Maruyama I, Nakajima T.
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Journal Title
Int J Mol Med
Volume: 24
Pages: 605-611
Related Report
Peer Reviewed
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