Construction of prion disease model using neuroblastoma cells and elucidation of the production mechanism of abnormal prion protein
| Project/Area Number |
18K07499
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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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| Review Section |
Basic Section 52020:Neurology-related
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| Research Institution | The University of Tokushima |
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Principal Investigator |
HARA Hideyuki 徳島大学, 先端酵素学研究所(次世代), 助教 (40469953)
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| Project Period (FY) |
2018-04-01 – 2021-03-31
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| Project Status |
Completed (Fiscal Year 2020)
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| Budget Amount *help |
¥4,420,000 (Direct Cost: ¥3,400,000、Indirect Cost: ¥1,020,000)
Fiscal Year 2020: ¥1,300,000 (Direct Cost: ¥1,000,000、Indirect Cost: ¥300,000)
Fiscal Year 2019: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
Fiscal Year 2018: ¥1,560,000 (Direct Cost: ¥1,200,000、Indirect Cost: ¥360,000)
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| Keywords | プリオン / プリオン病 / ウイルス / ウイルス感染 / 神経変性 / 構造変換 / 異常型 |
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| Outline of Final Research Achievements |
Prion disease is a neurodegenerative disease in which normal prion protein expressed in healthy nerve tissues undergoes conformational conversion into an infectious, protease K resistant, abnormal prion protein that accumulates in the central nervous system. Idiopathic prion diseases, which account for about 77% of all human prion diseases, occur in one out of one million people each year, but their etiology are unknown and there is no cure. In this study, we infected mouse neurons with neuropathogenic influenza A virus (IAV) in the hope that IAV infection could conformational conversion from normal prion protein into abnormal prion protein. As a result, we found that the normal prion protein undergoes conformational conversion into the abnormal prion protein.
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| Academic Significance and Societal Importance of the Research Achievements |
プリオン病は、病因が不明で治療法も存在しない希少疾患であるが、潜在的リスク保有者が多数おり、治療・予防法の確立が求められている。神経細胞にIAVを感染することで、正常型プリオンが異常型プリオンへと構造変換するという研究代表者の発見は、これまで不明だったプリオン病発症のトリガー因子としてIAV感染が関与している可能性を強く示しており、プリオン病の病因解明に新たな指針を与える。また、アルツハイマー病やパーキンソン病といった、蛋白質凝集体の蓄積が原因の神経変性疾患でも、ウイルス感染が凝集体形成のきっかけになる可能性が示唆されており、神経変性疾患全般の治療・予防に向けたフィードバックも可能となる。
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Report
(4 results)
Research Products
(10 results)
