トップ研究者を探す筋形成・維持におけるフクチン依存ジストログリカン糖鎖の役割と筋ジストロフィー病態

筋形成・維持におけるフクチン依存ジストログリカン糖鎖の役割と筋ジストロフィー病態

KAKEN 科学研究費助成事業データベース で見る
研究課題番号 KAKENHI-PROJECT-21790318
研究種目 若手研究(B)
研究分野 生物系
医歯薬学
基礎医学
病態医化学
研究機関 神戸大学
代表研究者 金川 基
研究期間 開始年月日 2008/4/1
研究期間 終了年度 2010
研究ステータス 完了 (2010/4/1)
配分額(合計) 4,290,000 (直接経費 :3,300,000、間接経費 :990,000)
配分額(履歴) 2010年度:1,820,000 (直接経費 :1,400,000、間接経費 :420,000)
2009年度:2,470,000 (直接経費 :1,900,000、間接経費 :570,000)
キーワード 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
筋形成・維持

研究成果

[学会発表] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.

Kanagawa M 2010

[学会発表] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.

Kanagawa M 2010

[学会発表] Molecular basis and physiological roles of dystroglycan glycosylation and its relevance to diseases

Motoi Kanagawa 2010

[雑誌論文] Residual glycosylation of dystroglycan in model mice to Fukuyama-type congenital muscular dystrophy is sufficient to maintain voluntary exercise activity and skeletal muscle regeneration capability.

Lu Z, Kanagawa M, Toda T. 2010

[学会発表] レトロトランスポゾン挿入変異をもつ福山型先天性筋ジストロフィーモデルマウスの創出と糖鎖遺伝子治療

金川基 2010

[学会発表] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy

Motoi Kanagawa 2010

[雑誌論文] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.

Motoi Kanagawa 2010

[学会発表] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy

Motoi Kanagawa 2010

[雑誌論文] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization.

Kanagawa M, Omori Y, Sato S, Kobayashi K, Miyagoe-Suzuki Y, Takeda S, Endo T, Furukawa T, Toda T. 2010

[学会発表] Post-translational maturation of dystroglycan is necessary for pikachurin binding and ribbon synaptic localization

Motoi Kanagawa 2010

[学会発表] Molecular basis and physiological roles of dystroglycan glycosylation and its relevance to diseases

Kanagawa M 2010

[学会発表] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy

Motoi Kanagawa 2010

[学会発表] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy.

Kanagawa M 2010

[学会発表] Disruption of dystroglycan-pikachurin interaction underlies the molecular pathogenesis of eye abnormalities in dystroglycanopathy.

Kanagawa M 2010

[学会発表] レトロトランスポゾン挿入変異をもつ福山型先天性筋ジストロフィーモデルマウスの創出と糖鎖遺伝子治療

金川基 2010

[学会発表] Essential role of dystroglycan in the maintenance of cell membrane.

Kanagawa M 2009

[雑誌論文] Basal lamina strengthens cell membrane integrity via the laminin G domain-binding motif of alpha-dystroglycan.

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. 2009

[雑誌論文] Residual laminin-binding activity and enhanced dystroglycan glycosylation by LARGE in novel model mice to dystroglycanopathy.

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. 2009

[学会発表] Subcellular localization and POMGnT1-binding of fukutin missense mutants which are involved in the onset of FCMD.

Kanagawa M 2009

[学会発表] ジストログリカンの糖鎖異常を示すモデルマウスを用いた先天性筋ジストロフィーの病態解析と治療戦略の構築

金川基 2008

[雑誌論文] Pikachurin, a dystroglycan ligand, is essential for photoreceptor ribbon synapse formation.

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. 2008

[学会発表] Generation of a model mouse for Fukuyama congenital muscular dystrophy carrying a retrotransposal insertion in the 3' UTR in the fukutin gene.

Kanagawa M 2008

[学会発表] ジストログリカンの糖鎖異常を伴う先天性筋ジストロフィーの病態と治療戦略

金川基 2008

[雑誌論文] Reduced expression of sarcospan in muscles of Fukuyama congenital muscular dystrophy.

Wakayama Y, Inoue M, Kojima H, Yamashita S, Shibuya S, Jimi T, Hara H, Matsuzaki Y, Oniki H, Kanagawa M, Kobayashi K, Toda T. 2008