Elucidation of the molecular mechanism of sarcolemmal repair system for the therapy for dysferlinopathy
| Project/Area Number |
21591105
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Neurology
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| Research Institution | National Institute of Advanced Industrial Science and Technology |
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Principal Investigator |
MATSUDA Chie 独立行政法人産業技術総合研究所, バイオメディカル研究部門, 主任研究員 (50344099)
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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: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2010: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2009: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | 脳神経疾患 / タンパク質 / 生体分子 / 筋疾患 / 筋ジストロフィー / 細胞膜修復 / ディスフェルリン / 骨格筋 / アフィキシン(β-パルビン) / 膜修復 |
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| Research Abstract |
Dysferlin is involved in plasma membrane repair in skeletal muscle, and mutations in the dysferlin gene cause muscular dystrophy(Miyoshi Myopathy and LGMD2B). We examined the possibility whether dysferlin-binding protein(MG53, caveolin-3 and affixin/β-parvin) particioate in sarcolemmal repair by membrane-repair assay using two-photon laser microscope. We found that MG53 and affixin accumulate at the wounded site of sarcolemma. This result suggests the involvement of MG53 and affixin in sarcolemmal repair.
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Report
(4 results)
Research Products
(15 results)
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[Journal Article] Human PTRF mutations cause secondary deficiency of caveolins resulting in muscular dystrophy with generalized lipodystrophy2009
Author(s)
Hayashi YK, Matsuda C, Ogawa M, Goto K, Tominaga K, Mitsuhashi S, Park YE, Nonaka I, Hino-Fukuyo N, Haginoya K, Sugano H, Nishino I
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Journal Title
J Clin Invest.
Volume: 119(9)
Issue: 9
Pages: 2623-33
DOI
Related Report
Peer Reviewed
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