Elucidation of the molecular mechanism of sarcolemmal repair system for the therapy for dysferlinopathy

• Project/Area Number: 21591105
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Single-year Grants
• Section: 一般
• Research Field: Neurology
• Research Institution: National Institute of Advanced Industrial Science and Technology
• 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)
• Project Period (FY): 2009 – 2011
• Project Status: Completed (Fiscal Year 2011)
• Budget Amount: ¥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)
• 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

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.

Research Products

• [Journal Article] Augmented autocrine bone morphogenic protein(BMP) 7 signaling increases the metastatic potential of mouse breast cancer cells (2012)
  • Author(s): Sakai H, Furihata M, Matsuda C, Takahashi M, Miyazaki H, Konakahara T, Imamura T, Okada T.
  • Journal Title: Clin Exp Metastasis Volume: 29(4) Pages: 327-38
  • Peer Reviewed
• [Journal Article] Augmented autocrine bone morphogenic protein (BMP) 7 signaling increases the metastatic potential of mouse breast cancer cells (2012)
  • Author(s): Hirofumi Sakai, Mutsuo Furihata, Chie Matsuda, Munehisa Takahashi, Hiroshi Miyazaki, Takeo Konakahara, Toru Imamura
  • Journal Title: CLINICAL & EXPERIMENTAL METASTASIS Volume: 29 Issue: 4 Pages: 327-338
  • DOI: 10.1007/s10585-012-9453-9
  • Peer Reviewed
• [Journal Article] Specific phosphorylation on Ser458 of A-type lamins in LMNA associated myopathy patients (2010)
  • Author(s): Mitsuhashi H, Hayashi YK, M Noguchi S, Wakatsuki S, Araki T, and Nishino I
  • Journal Title: J Cell Sci. Volume: 123(22) Pages: 3893-900
  • Peer Reviewed
• [Journal Article] Specific phosphorylation of Ser458 of A-type lamins in LMNA-associated myopathy patients. (2010)
  • Author(s): Mitsuhashi H, Hayashi YK, Matsuda C, Noguchi S, Wakatsuki S, Araki T, Nishino I.
  • Journal Title: Journal of Cell Science Volume: 123(Pt 22) Pages: 3893-3900
  • Peer Reviewed
• [Journal Article] Human PTRF mutations cause secondary deficiency of caveolins resulting in muscular dystrophy with generalized lipodystrophy (2009)
  • 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
  • Journal Title: J Clin Invest. Volume: 119(9) Issue: 9 Pages: 2623-33
  • DOI: 10.1172/jci38660
  • Peer Reviewed
• [Journal Article] Defective myotilin homodimerization caused by a novel mutation in MYOT exon 9 in the first Japanese limb girdle muscular dystrophy 1A patient. (2009)
  • Author(s): Shalaby S, Mitsuhashi H, Matsuda C, et al
  • Journal Title: J Neuropathol Exp Neurol. 68 Pages: 701-707
  • Peer Reviewed
• [Journal Article] Human PTRF mutations cause secondary deficiency of caveolins resulting in muscular dystrophy with generalized lipodystrophy. (2009)
  • Author(s): Hayashi YK, Matsuda C, et al
  • Journal Title: J Clin Invest. 119 Pages: 2623-2633
  • Peer Reviewed
• [Presentation] Real time analysis of sarcolemmal repair mediated by dysferlin and MG53 (2011)
  • Author(s): Chie Matsuda, Katsuya Miyake, Kimihiko Kameyama, Hiroshi Takeshima, Etsuko Keduka, Nobukazu Arakai, Ichizo Nishino and Yukiko K. Hayashi
  • Organizer: 16th International WMS Congress
  • Place of Presentation: Ria Park Hotel, Almancil, Algarve, Portugal
  • Year and Date: 2011-10-19
• [Presentation] Real time analysis of sarcolemmal repair mediated by dysferlin and MG53 (2011)
  • Author(s): 松田知栄、三宅克也、亀山仁彦, 他
  • Organizer: the 16th International WMS Congress
  • Place of Presentation: Algarve, Portugal
  • Year and Date: 2011-10-19
• [Presentation] Dysferlin C2A domain is important for association with MG53(TRIM72) (2010)
  • Author(s): Chie Matsuda, Kimihiko Kameyama, Hiroshi Takeshima, Ichizo Nishino and Yukiko K. Hayashi
  • Organizer: The XII International Congress of Neuromuscular Dystrophy
  • Place of Presentation: Monte S. Angelo University Campus, Naples, Italy
  • Year and Date: 2010-07-19
• [Presentation] Dysferlin C2A domain is important for association with MG53 (TRIM72) (2010)
  • Author(s): 松田知栄、亀山仁彦、竹島浩、西野一三、林由起子
  • Organizer: 12th International Congress on Neuromuscular Diseases
  • Place of Presentation: Naples(Italy)
  • Year and Date: 2010-07-19
• [Presentation] Dysferlin C2A domain is important for association with MG53(TRIM72) (2010)
  • Author(s): Chie Matsuda, Katsuya Miyake, Kimihiko Kameyama, Hiroshi Takeshima, Nobukazu Arakai, Ichizo Nishino and Yukiko K. Hayashi
  • Organizer: 9^<th> Annual Meeting of Asian and Oceanian Myology Center
  • Place of Presentation: Millennium Seoul Hilton hotel, Seoul, Korea
  • Year and Date: 2010-03-25
• [Presentation] Dysferlin C2A domain is important for association of MG53(TRIM72). (2010)
  • Author(s): 松田知栄
  • Organizer: 9^<th> AOMC Myology Center Scientific Meeting
  • Place of Presentation: Seoul, Korea
  • Year and Date: 2010-03-25
• [Presentation] The association of dysferlin and affixin is regulated by calcium concentration (2009)
  • Author(s): Chie Matsuda, Katsuya Miyake, Kimihiko Kameyama, Hiroshi Takeshima, Nobukazu Arakai, Ichizo Nishino and Yukiko K. Hayashi
  • Organizer: 14th International WMS Congress
  • Place of Presentation: Geneva, Switzerland
  • Year and Date: 2009-09-10
• [Presentation] The association of dysferlin and affixin is regulated by calcium concentration. (2009)
  • Author(s): 松田知栄
  • Organizer: 14^<th> World Muscle Society
  • Place of Presentation: Geneva, Switzerland
  • Year and Date: 2009-09-10