2019 Fiscal Year Final Research Report
Study of the pathogenesis of west syndrome using a new model rat
Project/Area Number |
16H05354
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Research Category |
Grant-in-Aid for Scientific Research (B)
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Allocation Type | Single-year Grants |
Section | 一般 |
Research Field |
Pediatrics
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Research Institution | Okayama University |
Principal Investigator |
Ouchida Mamoru 岡山大学, 医歯薬学総合研究科, 准教授 (80213635)
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Co-Investigator(Kenkyū-buntansha) |
大守 伊織 岡山大学, 教育学研究科, 教授 (20403488)
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Project Period (FY) |
2016-04-01 – 2020-03-31
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Keywords | 発達性およびてんかん性脳症 / 疾患モデルラット / 酸化ストレス |
Outline of Final Research Achievements |
On the study of mutant rats established by ENU mutagenesis, we found a lineage of diseased rats with developmental and epileptic encephalopathy-like epilepsy. Epileptic seizures occurred during a limited period of 4 to 6 weeks of age. The brain lesions were recovered spontaneously. The causative gene found in the rats is a missense mutation of a gene that regulates oxidative stress by redox reaction. Our results suggested that there may be a critical period when the oxidative stress becomes particularly high in the local region of the brain during the developmental process of the brain. Mutant rats may have reduced ability to properly suppress the oxidative stress due to its low function of antioxidant activity, resulting in damage of the brain.
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Free Research Field |
分子生物学
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Academic Significance and Societal Importance of the Research Achievements |
酸化ストレスを制御する働きを持つ遺伝子の変異により、発達性およびてんかん性脳症に酷似した症状が自然に発症するという現象は世界初の発見であり、非常に革新的である。当疾患ラットの発症メカニズム解明は他に類を見ない研究であり、難病疾患モデル動物としても研究発展が見込まれ、国内外への大きな波及効果が期待される。当該モデル動物を用いた発達性およびてんかん性脳症の治療法開発は、難治てんかんのみならず他の酸化ストレス関連疾患の治療技術開発にも繋がると期待される。
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