2013 Fiscal Year Final Research Report
Study on relation between acute encephalopathy in childhood and causative disorders of organic and fatty acid metabolism
| Project/Area Number |
22390208
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| Research Category |
Grant-in-Aid for Scientific Research (B)
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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 | Shimane University |
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Principal Investigator |
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| Co-Investigator(Renkei-kenkyūsha) |
FUKAO Toshiyuki 岐阜大学, 大学院・医学系研究科, 教授 (70260578)
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| Project Period (FY) |
2010-04-01 – 2014-03-31
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| Keywords | ミトコンドリアβ酸化 / 脂肪酸代謝異常症 / 急性脳症 / in vitro probe assay / 分子解析 / ベザフィブラート / 細菌毒素 / 環境温度 |
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| Research Abstract |
Acute encephalopathy of various causes like hyperpyrexia or infection is not rare in childhood. We studied the relation between acute encephalopathy and disorders of organic and fatty acid metabolism to develop the biochemical and molecular diagnostic system, and made a diagnosis on over 74 cases with disorders of organic and fatty acid metabolism.
We investigated the mechanism of acute encephalopathy in case of food poisoning in view of fatty acid oxidation impairment, and found that the acute encephalopathy was caused by temporary disturbance of fatty acid oxidation by the toxin. Furthermore, we developed a new therapy with bezafibrate for fatty acid oxidation disorders, using the in vitro probe assay, using cultured cells and tandem mass spectrometry.
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