| Project/Area Number |
26461550
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| Research Category |
Grant-in-Aid for Scientific Research (C)
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| Allocation Type | Multi-year Fund |
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| Section | 一般 |
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| Research Field |
Pediatrics
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| Research Institution | Osaka City University |
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Principal Investigator |
SETO Toshiyuki 大阪市立大学, 大学院医学研究科, 講師 (60423878)
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| Co-Investigator(Kenkyū-buntansha) |
田中 あけみ 大阪市立大学, 大学院医学研究科, 准教授 (30145776)
濱崎 考史 大阪市立大学, 大学院医学研究科, 准教授 (40619798)
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| Project Period (FY) |
2014-04-01 – 2018-03-31
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| Project Status |
Completed (Fiscal Year 2017)
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| Budget Amount *help |
¥5,070,000 (Direct Cost: ¥3,900,000、Indirect Cost: ¥1,170,000)
Fiscal Year 2016: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2015: ¥1,690,000 (Direct Cost: ¥1,300,000、Indirect Cost: ¥390,000)
Fiscal Year 2014: ¥2,470,000 (Direct Cost: ¥1,900,000、Indirect Cost: ¥570,000)
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| Keywords | ムコ多糖症 / サイトカイン / プテリジン / 自閉症 / 知的障害 / 遺伝学的未診断 / 免疫不全 / 慢性炎症 / 先天代謝異常症 / 髄膜炎 / ネオプテリン / ムンプスウイルス / エンテロウイルス / iPS細胞 |
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| Outline of Final Research Achievements |
Neuroglial complications, which are intellectual disability (ID) and autistic disorder, of congenital mucoporysaccharidosis (MPS) are speculated to be related with chronic inflammation in central nervous system. We have studied the pathological mechanism compared with various children with autism and ID. In order to evaluate inflammation on MPS patient we performed analysis of pteridine derivatives and cytokines, it clarified elevated some markers of inflammation. On the other hand, in order to study pathology of MPS brain, we used MPS type II model mice (IDS-KO). It was revealed that there were vacuoles in various type of cells in the brain by electron microscopy and immunostaining related autophagy. It was effective to administrate drug for suppression of autophagy in IDS-KO pathologically. Longitudinal study of MRI on MPS type II, it was shown that hematopoietic stem cell transplantation was effective for brain to some extent.
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