| Project/Area Number |
16590859
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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 |
Neurology
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| Research Institution | Shizuoka Institute of Epilepsy and Neurological Disorders |
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Principal Investigator |
MIZOGUCHI Koichi Shizuoka Institute of Epilepsy and Neurological Disorders, Department of Research, Researcher, てんかん神経医療センター臨床研究部)・遺伝子生化学研究室, 室員 (50190666)
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| Co-Investigator(Kenkyū-buntansha) |
TAKAHASHI Yukitoshi Shizuoka Institute of Epilepsy and Neurological Disorders, National Epilepsy Center, Department of Research, Senior Director of Research, てんかん神経医療センター臨床研究部), 部長 (70262764)
TANAKA Masaki Shizuoka Institute of Epilepsy and Neurological Disorders, National Epilepsy Center, Department of Research, Director of Research, てんかん神経医療センター臨床研究部)・薬理研究室, 室長 (90360809)
OBI Tomokazu Shioka Institute of Epilepsy and Neurological Disorders, Department of Research, Researcher, てんかん神経医療センター臨床研究部)・遺伝子生化学研究室, 室員 (10393117)
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| Project Period (FY) |
2004 – 2005
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| Project Status |
Completed (Fiscal Year 2005)
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| Budget Amount *help |
¥3,100,000 (Direct Cost: ¥3,100,000)
Fiscal Year 2005: ¥700,000 (Direct Cost: ¥700,000)
Fiscal Year 2004: ¥2,400,000 (Direct Cost: ¥2,400,000)
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| Keywords | spinocerebellar degeneration / DRPLA / GluRε2 / GluRδ / neuronal death / multiple system atrophy / cortex cerebellar atrophy / 慢性小脳炎 / 脳幹脳炎 / グルタミン酸受容体 / 自己免疫 / オプソクローヌス・ミオクローヌス症候群 / 小脳失調と毛細血管拡張を伴う免疫不全症 |
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| Research Abstract |
We examined autoantibodies against GluRε2 and GluRδ2 in patients with spinocerebellar degeneration, to reveal the mechanisms of neuronal apoptosis and to establish novel treatments.
1.Autoantibodies against GluRs in patients with solitary spinocerebellar degeneration
We examined autoantibodies against GluRε2 and GluRδ2 in sera and CSF from 12 patients with solitary spinocerebellar degeneration. Autoantibodies against GluRδ2 were detected in sera from 60% of patients and in CSF from 70% of patients. IgM-autoantibodies against GluRδ2 were detected in CSF more frequently in patients with multiple system atrophy than in those with cortex cerebellar atrophy. Autoantibodies against GluRε2 were detected in sera from all patients and in CSF from 60% of patients. Autoantibodies against GluRε2 were not detected in CSF from patients with cortex cerebellar atrophy, and were detected in CSF from 60% of patients with multiple system atrophy.
These data suggest that patients with multiple system atrophy
… More
might have more severe neuronal death of cerebellar Purkinje cells and cerebral involvement, comparing with patients of cortex cerebellar atrophy.
2.Autoantibodies against GluRs in patients with hereditary spinocerebellar degeneration
We examined autoantibodies against GluRε2 and GluRδ2 in sera and CSF from 10 patients with hereditary spinocerebellar degeneration. Autoantibodies against GluRδ2 were detected in sera from 75% of patients and in CSF from 70% of patients. Autoantibodies against GluRδ2 appeared in a patient with DRPLA, evolutionally after the onset. Autoantibodies against GluRε2 were detected in sera from 50% of patients and in CSF from 55% of patients. Autoantibodies against GluRε2 were detected frequently in CSF from patients with DRPLA (80%), but rarely in CSF from patients with hereditary spinocerebellar degeneration other than DRPLA (25%).
These data suggest that autoantibodies against GluRδ2 might be produced secondarily after the onset of DRPLA. Autoantibodies against GluRε2 in CSF might contribute to the mental impairment and cognitive dysfunction. Less
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