Studies of functional glycans on alfa-dystroglycan associated with congenital muscular dystrophies
Project/Area Number |
26291021
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Partial Multi-year Fund |
Section | 一般 |
Research Field |
Functional biochemistry
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Research Institution | Kyoto University |
Principal Investigator |
Oka Shogo 京都大学, 医学(系)研究科(研究院), 教授 (60233300)
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Co-Investigator(Kenkyū-buntansha) |
竹松 弘 京都大学, 医学(系)研究科(研究院), 准教授 (80324680)
森瀬 譲二 京都大学, 医学(系)研究科(研究院), 助教 (60755669)
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Project Period (FY) |
2014-04-01 – 2017-03-31
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Project Status |
Completed (Fiscal Year 2016)
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Budget Amount *help |
¥16,120,000 (Direct Cost: ¥12,400,000、Indirect Cost: ¥3,720,000)
Fiscal Year 2016: ¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2015: ¥4,550,000 (Direct Cost: ¥3,500,000、Indirect Cost: ¥1,050,000)
Fiscal Year 2014: ¥7,020,000 (Direct Cost: ¥5,400,000、Indirect Cost: ¥1,620,000)
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Keywords | ジストログリカン / 先天性筋ジストロフィー / O-マンノース型糖鎖 / 糖転移酵素 / ラミニン結合性糖鎖 / ラミニン / POMGnT2/AGO61 / 神経初期発生 / ポストリン酸糖鎖 |
Outline of Final Research Achievements |
α-Dystroglycan (α-DG) is a cell surface glycoprotein that serves as a receptor for extracellular matrix components such as laminin. The α-DG-ligand interaction is mediated by a unique O-mannose-linked glycan attached to α-DG, and the glycosylation deficiency of α-DG causes several forms of congenital muscular dystrophies associated with cobblestone lissencephaly, classified as dystroglycanopathy. In this study, we investigated the regulatory mechanism for the unique O-mannose-linked glycan expression. We also revealed initial pathological events in the developing brain of Pomgnt2-knockout mouse as a dystroglycanopathy model.
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Report
(4 results)
Research Products
(33 results)
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[Journal Article] Psychosine-triggered endomitosis is modulated by membrane sphingolipids through regulation of phosphoinositide 4,5-bisphosphate production at the cleavage furrow.2016
Author(s)
Watanabe H, Okahara K, Naito-Matsui Y, Abe M, Go S, Inokuchi J, Okazaki T,Kobayashi T, Kozutsumi Y, Oka S, Takematsu H.
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Journal Title
Mol Biol Cell.
Volume: 27
Issue: 13
Pages: 2037-2050
DOI
Related Report
Peer Reviewed / Open Access
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[Journal Article] A sulfated glycosaminoglycan linkage region is a novel type of Human Natural Killer-1 (HNK-1) epitope expressed on aggrecan in perineuronal nets.2015
Author(s)
Yabuno, K., Morise, J., Kizuka, Y., Hashii, N., Kawasaki N., Takahashi, S., Miyata, S., Izumikawa, T., Kitagawa, H., Takematsu, H., and Oka, S.
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Journal Title
PLoS ONE,
Volume: 10
Issue: 12
Pages: e0144560-e0144560
DOI
NAID
Related Report
Peer Reviewed / Open Access
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[Journal Article] Loss of α1,6-fucosyltransferase decreased hippocampal long-term potentiation: implications for core fucosylation in the regulation of AMPA receptor heteromerization and cellular signaling.2015
Author(s)
Gu, W., Fukuda, T., Isaji, T., Hang, Q., Lee, H., Sakai, S., Morise, J., Mitoma, J., Higashi, H., Taniguchi, N., Yawo, H., Oka, S. and Gu, J.
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Journal Title
J. Biol. Chem.
Volume: 290
Issue: 28
Pages: 17566-75
DOI
Related Report
Peer Reviewed / Open Access / Int'l Joint Research
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[Book] Glycoscience: Biology and Medicine (Taniguchi, N., Endo, T., Hart, G.W., Seeberger, P.H., and Wong, C.H., eds)2015
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