| Project/Area Number |
21591322
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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 |
Pediatrics
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| Research Institution | Osaka University |
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Principal Investigator |
SAKAI Norio 大阪大学, 医学系・研究科, 准教授 (30314313)
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| Co-Investigator(Kenkyū-buntansha) |
TANIIKE Masako 大阪大学, 連合小児発達学研究科, 教授 (30263289)
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| Project Period (FY) |
2009 – 2011
|
| Project Status |
Completed (Fiscal Year 2011)
|
| Budget Amount *help |
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2011: ¥650,000 (Direct Cost: ¥500,000、Indirect Cost: ¥150,000)
Fiscal Year 2010: ¥910,000 (Direct Cost: ¥700,000、Indirect Cost: ¥210,000)
Fiscal Year 2009: ¥3,250,000 (Direct Cost: ¥2,500,000、Indirect Cost: ¥750,000)
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| Keywords | ムコリピドーシス / I-cell病 / G1cNAc-transphosphatase / リソソーム病 / 病態解析 / 治療実験 / G1cNAc-phosphotranspharase / ライソゾーム病 / ライソゾームのpH / GlcNac-phosphotranspherase / GlcNAc-transphosphatase |
|---|
| Research Abstract |
Mucolipidosis is one of the lysosomal storage diseases that the transport of most of the lysosomal enzymes to the lysosome is affected, however the cellular pathology of the disease is not elucidated. And the treatment such as enzyme replacement therapy for some lysosomal diseases is not established. On this research we aimed to clarify the cellular pathology in the patient skin fibroblasts and found new pathology. We also analyzed the cellular effect of the mixed lysosomal enzymes which are extracted from the normal cells with NH4Cl, and the many cellular effects were proved.
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