Development of innovative non-invasive diagnostic methods for prion disease

2020 Fiscal Year Final Research Report

• Project/Area Number: 17K09759
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Nagasaki University
• Principal Investigator: SATOH Katsuya 長崎大学, 医歯薬学総合研究科(保健学科), 教授 (70398147)
• Project Period (FY): 2017-04-01 – 2021-03-31
• Keywords: 神経変性疾患 / プリオン病 / 異常プリオン蛋白の半定量化 / 臓器 / RT-QuIC法

Outline of Final Research Achievements

Human prion disease (HPD) is 100% lethal rapidly progressive dementia, and no diagnostic or therapeutic method for HPD has yet been established. We have succeeded for the first time in the world in detecting the abnormal prion proteins in the CSF of patients with HPD. The purpose of this study is to attempt an innovative and non-invasive diagnosis of HPD from non-CNS organs containing blood ahead of the rest of the world. From 2017 to March　2021 , based on the research results so far, an epoch-making diagnostic method was established based on four studies. （1） Establish semi-quantification of trace amounts of abnormal prion protein, especially in human samples. (2)Analysis of stored samples and 4-year retrospective study will be conducted to establish sensitivity and specificity for diagnosis from CSF, and semi-quantification will also be performed. (3) We developed a new detection method for abnormal prion proteins from samples of HPD patients, and examined its effectiveness.

Free Research Field

神経疾患、神経内科学

Academic Significance and Societal Importance of the Research Achievements

プリオン病は100%の致死性急速進行性認知症であり、プリオン病の診断法・治療法は未だ確立されていない。我々はプリオン病患者髄液中の超微量の異常プリオン蛋白の検出に世界で初めて成功した。本研究は世界に先んじて血液を含んだ非中枢神経の臓器からのプリオン病の革新的かつ非侵襲的な診断を試みた。プリオン病患者のサンプル（血清・各種臓器）からの異常プリオン蛋白の新規検出法を開発し、その有効性について検討を行った。特に非侵襲性組織からプリオン活性の検出法に成功し、プリオン病の治験の実施が可能になれば実験計画の達成への期待が高まる。