2011 Fiscal Year Final Research Report
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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| Keywords | 脳神経疾患 / タンパク質 / 生体分子 |
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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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[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)
Pages: 2623-33
DOI
Peer Reviewed
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