| Project/Area Number |
11670195
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Single-year Grants |
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| Section | 一般 |
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| Research Field |
Human pathology
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| Research Institution | Tokyo Women's Medical University |
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Principal Investigator |
YAMAMOTO Tomoko Tokyo Women's Medical University, Pathology, Assistant professor, 医学部, 講師 (10239634)
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| Co-Investigator(Kenkyū-buntansha) |
KOBAYASHI Makio Tokyo Women's Medical University, Pathology, Professor, 医学部, 教授 (80060086)
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| Project Period (FY) |
1999 – 2000
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| Project Status |
Completed (Fiscal Year 2000)
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| Budget Amount *help |
¥3,500,000 (Direct Cost: ¥3,500,000)
Fiscal Year 2000: ¥2,300,000 (Direct Cost: ¥2,300,000)
Fiscal Year 1999: ¥1,200,000 (Direct Cost: ¥1,200,000)
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| Keywords | muscular dystrophy / Fukuyama type / astrocyte / oxidative modification endoproducts / superoxide dismutase / 福山型先天性筋ジストロフィー / advanced glycation endproducts / 4-hydroxynonenala / 8-hydroxy deoxyguanosine / CuZnsuperoxide disumtase / Mn superoxide dismutase |
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| Research Abstract |
Disruption of the glia limitans due to abnormal basement membrane has been considered to be involved in the genesis of the brain lesion of Fukuyama congenital muscular dystrophy (FCMD). The glia limitans is formed by astrocytic endfeet and is covered with the basement membrane. We reported previously that immature astrocytes in the FCMD brain may not be able to participate in the function of the basement membrane. Immunohistochemical examination for oxidative modification endproducts was performed in the cerebrum and medulla oblongata of 8 FCMD cases including 2 fetal cases to elucidate the characteristics of astrocytes in the FCMD brain. Non-FCMD cases including 3 fetal cases were used for controls. In fetal cases, significant difference was not found between FCMD and control cases. In cases of children and adults, advanced glycation endproduct tended to be weakly positive in cerebral astrocytes of FCMD cases, although it was negative in most of the control cases. Difference was not found in other oxidative modification endoproducts such as 4-hydroxynonenal and 8-hydroxy deoxyguanosine, or endproducts formed by non-oxidative pathway such as pyrraline and methylglyoxal. Astrocytes positive for Mn superoxide dismutase tended to increase in FCMD cases. Our data suggest that astrocytes in FCMD, which seems to play a significant role for formation of the brain lesion, is sensitive to oxidative stress and Mn SOD shows reactive increase against oxidative stress. Quantitative analysis such as Western blot hybridization will be performed with more accumulation of cases.
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