Study on mechanisms of inflammation in the central nervous system of gangliosidosis
Project/Area Number |
23590468
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Research Category |
Grant-in-Aid for Scientific Research (C)
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Allocation Type | Multi-year Fund |
Section | 一般 |
Research Field |
Experimental pathology
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Research Institution | Yokohama City University |
Principal Investigator |
YAMANAKA Shoji 横浜市立大学, 附属病院, 准教授 (80264604)
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Co-Investigator(Kenkyū-buntansha) |
YAMAGUCHI Akira 横浜市立大学, 医学研究科, 客員研究員 (20381585)
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Project Period (FY) |
2011 – 2013
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Project Status |
Completed (Fiscal Year 2013)
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Budget Amount *help |
¥5,330,000 (Direct Cost: ¥4,100,000、Indirect Cost: ¥1,230,000)
Fiscal Year 2013: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2012: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2011: ¥2,860,000 (Direct Cost: ¥2,200,000、Indirect Cost: ¥660,000)
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Keywords | GM2ガングリオシドーシス / サンドホフ病 / Toll like receptor / 炎症 / ライソゾーム病 / GM2ガングリオーシド / CXCL-13 / BAFF / 炎症反応 / 自己抗体 / ガングリオシド |
Research Abstract |
Sandhoff disease is a lysosomal storage disorder characterized by the absence of beta-hexosaminidase and strage of GM2 gangliodside and related glycolipid. Recently we and others have reported several immunological abnormalities in the CNS. However, little is known about the mechanism underlying the action of accumulation Gmix as a leading cause for pathological inflammatory responses in the brain. Here we reported that autoantibodies and released-Gmix from apoptotic neuronal cells mediate the nervous inflammation in microglia and astorocytes. The Gmix storage of neuronal cells in SD mice can lead to the release of Gmix from Apoptotic neuronal cells into the extracellular space. The exposure to Gmix can induce the production of TNF-alpha, IL-6 and MIP-1alpha via the Toll-like receptor 4 in the SD mice microglia and astorocytes.Taken together, these results suggest that autoantibodies and released-Gmix may provide to cause nervous inflammatory condition in the brain of SD mice.
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Report
(4 results)
Research Products
(18 results)
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[Journal Article] 維持透析患者に対して術中血液濾過透析を施行した腹腔鏡下褐色細胞腫摘出術の1例.2012
Author(s)
大竹慎二, 涌井広道, 垣本みどり, 橋本達夫, 田村功一, 戸谷義幸, 槙山和秀, 坂田綾子, 蓼沼知之, 窪田吉信, 山中正二, 梅村敏.
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