| Project/Area Number |
15H04352
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Functional biochemistry
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| Research Institution | Kobe University |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
片野坂 友紀 岡山大学, 医歯薬学総合研究科, 助教 (60432639)
山口 芳樹 国立研究開発法人理化学研究所, 主任研究員研究室等, チームリーダー (90323451)
永森 收志 大阪大学, 医学系研究科, 准教授 (90467572)
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| Project Period (FY) |
2015-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥16,120,000 (Direct Cost: ¥12,400,000、Indirect Cost: ¥3,720,000)
Fiscal Year 2017: ¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2016: ¥5,460,000 (Direct Cost: ¥4,200,000、Indirect Cost: ¥1,260,000)
Fiscal Year 2015: ¥5,980,000 (Direct Cost: ¥4,600,000、Indirect Cost: ¥1,380,000)
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| Keywords | 糖鎖 / 筋ジストロフィー / リビトールリン酸 / フクチン / 細胞・組織 / 糖鎖生物学 / ジストログリカン |
|---|
| Outline of Final Research Achievements |
Post-phosphoryl sugar chain is associated with muscular dystrophy and brain malformation and thus is believed to play important physiological roles. However, its structure, modification mechanism, and physiological functions are remained to be elucidated. In this study, we identified tandemly connected ribitol-phosphate, a novel post-modification unit in mammals, as a substance of post-phosphoryl modification. We also identified four enzymes involved in the biosynthesis of ribitol-phosphate modification. These enzymes are encoded by genes responsible for muscular dystrophy. Furthermore, we generated model mice for ribitol-phosphate deficiency and revealed pathomechamism of brain abnormality in muscular dystrophy patients, leading to a proposal of novel therapeutic strategies.
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