Evaluation of mitophagy activity in patients with Leigh encephalopathy
| Project/Area Number |
15K09597
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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 | Aichi Medical University (2018)
Oita University (2015-2017) |
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Principal Investigator |
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| Co-Investigator(Kenkyū-buntansha) |
井原 健二 大分大学, 医学部, 教授 (80294932)
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| Project Period (FY) |
2015-04-01 – 2019-03-31
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| Project Status |
Completed (Fiscal Year 2018)
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| Budget Amount *help |
¥4,680,000 (Direct Cost: ¥3,600,000、Indirect Cost: ¥1,080,000)
Fiscal Year 2017: ¥780,000 (Direct Cost: ¥600,000、Indirect Cost: ¥180,000)
Fiscal Year 2016: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Fiscal Year 2015: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
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| Keywords | ミトコンドリア病 / Leigh脳症 / ミトファジー / MELAS / 脳卒中様エピソード / 変異ミトコンドリア / ミトコンドリア活性 / オートファジー / 線維芽細胞 |
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| Outline of Final Research Achievements |
At first, we tried to culture fibroblast cells derived from patients with Leigh encephalopathy, but the fibroblasts were too fragile to continue passage culture. Next, we started to assess mutation rate of mitochondrial DNA and activivty of mitochondria in patients with MELAS, which was different type of mitochondrial disease. Recently, our report regarding MELAS/MERRF overlap syndrome was accepted in Neuropathology. In the future, we keep evatuating the experiments about MELAS, and submit a reserch article until 2020.
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| Academic Significance and Societal Importance of the Research Achievements |
ミトコンドリア病はミトコンドリアの機能異常が原因であると推察されているが、発症メカニズムについては十分に解明されておらず、治療も対症的なものしかない。発症にミトコンドリアDNAの変異率の上昇やミトコンドリアの機能異常が関連していることを明らかにできれば、遺伝子治療などの根本治療への足掛かりになり、将来的にはミトコンドリア病患者の根本的治療に貢献できる可能性がある。
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Report
(5 results)
Research Products
(1 results)
