2012 Fiscal Year Final Research Report
Analysis of brain infiltrating monocyteic cell in lysosomal disease
| Project/Area Number |
22790318
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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 | The University of Tokushima |
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Principal Investigator |
TSUJI Daisuke 徳島大学, 大学院・ヘルスバイオサイエンス研究部, 助教 (00423400)
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| Project Period (FY) |
2010 – 2012
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| Keywords | 先天性代謝異常症 / 糖脂質 / リソソーム病 / ケモカイン / 脳内浸潤 |
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| Research Abstract |
GM2 gangliosidosis is a lysosomal beta-hexosaminidase (Hex) deficiency involving excessive accumulation of undegraded substrates, including GM2 ganglioside (GM2), and progressive neurodegeneration. In this study, we isolated monocyte cell line from the bone marrow of Sandhoff disease model mice, and demonstrated the abnormalities of signal transduction in monocyte cell lines. We found remarkable differences in the cell migraion for MIP-1alpha of SD monocyte (MSD) when compared to cells isolated from wild type (WT) mice, with activation of MSD. In addition, we observed increased Cofilin phosphorylation in MSD cells. Moreover, PI3K activity was increased about 5times in MSD compare with that of WT. These results suggest that the up-regulation of PI3K of MSD was dependent upon accumulation of natural substrates, including GM2. These findings may represent a mechanism in linking infiltrating cells into brain observed in GM2 gangliosidosis.
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[Journal Article] Small molecule antibody targeting HLA class I inhibits myeloma cancer stem cells by repressing pluripotency-associated transcription factors2012
Author(s)
Ikegame A, Ozaki S, Tsuji D, Harada T, Fujii S, Nakamura S, Miki H, Nakano A, Kagawa K, Takeuchi K, Abe M, Watanabe K, Hiasa M, Kimura N, Kikuchi Y, Sakamoto A, Habu K, Endo M, Itoh K, Yamada-Okabe H, MatsumotoT
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Journal Title
Leukemia
Volume: 26
Pages: 2124-2134
DOI
Peer Reviewed
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[Journal Article] Highly phosphomannosylated enzyme replacement therapy for GM2 gangliosidosis2011
Author(s)
Tsuji D, Akeboshi H, Matsuoka K, Yasuoka H, Miyasaki E, Kasahara Y, Kawashima I, Chiba Y, Jigami Y, Taki T, Sakuraba H, Itoh K
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Journal Title
Ann Neurol
Volume: 69
Pages: 691-701
DOI
Peer Reviewed
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