2010 Fiscal Year Final Research Report
Role of fukutin-dependent glycosylation of dystroglycan on skeletal muscle development, maintenance, and muscular dystrophy pathogenesis
| Project/Area Number |
21790318
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| Research Category |
Grant-in-Aid for Young Scientists (B)
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| Allocation Type | Single-year Grants |
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| Research Field |
Pathological medical chemistry
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| Research Institution | Kobe University |
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Principal Investigator |
KANAGAWA Motoi Kobe University, 大学院・医学研究科, 助教 (00448044)
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| Project Period (FY) |
2008 – 2010
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| Keywords | フクチン / ジストログリカン / 福山型筋ジストロフィー / 糖鎖異常 / 筋形成維持 |
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| Research Abstract |
Fukuyama-type muscular dystrophy, which is caused by mutations in the fukutin gene, is a severe congenital form of muscular dystrophy. Pathogenesis of this disorder is poorly understood and currently no effective treatment is available. In this study, we generated conditional knock-out mice that lack fukutin selectively in the skeletal muscle. These mice showed pathology similar to Fukuyama-type muscular dystrophy, and thus would be a useful model for understanding molecular pathogenesis and establishing therapeutic strategy such as gene therapy.
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[Journal Article] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.2009
Author(s)
Kanagawa M, Nishimoto A, Chiyonobu T, Takeda S, Miyagoe-SuzukiY, Wang F, Fujikake N, Taniguchi M, Lu Z, Tachikawa M, Nagai Y, Tashiro F, Miyazaki J, Tajima Y, Takeda S, Endo T, Kobayashi K, Campbell KP, Toda T.
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Journal Title
Hum.Mol.Genet. 18
Pages: 621-631
Peer Reviewed
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[Journal Article] Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.2009
Author(s)
Han R, Kanagawa M, Yoshida-Moriguchi T, Rader EP, Ng RA, Michele DE, Muirhead DE, Kunz S, Moore SA, Iannaccone ST, Miyake K, McNeil PL, Mayer U, Oldstone MB, Faulkner JA, Campbell KP.
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Journal Title
Proc.Natl.Acad.Sci.USA. 106
Pages: 12573-12579
Peer Reviewed
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[Journal Article] Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation.2008
Author(s)
Sato S, Omori Y, Katoh K, Kondo M, Kanagawa M, Miyata K, Funabiki K, Koyasu T, Kajimura N, Miyoshi T, Sawai H, Kobayashi K, Tani A, Toda T, Usukura J, Tano Y, Fujikado T, Furukawa T.
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Journal Title
Nat.Neurosci. 11
Pages: 923-931
Peer Reviewed
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[Journal Article] Reduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophy.2008
Author(s)
Wakayama Y, Inoue M, Kojima H, Yamashita S, Shibuya S, Jimi T, Hara H, Matsuzaki Y, Oniki H, Kanagawa M, Kobayashi K, Toda T.
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Journal Title
Histol.Histopathol. 23
Pages: 1425-1438
Peer Reviewed
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