Rapid diagnosis of aqcuired Creutzfeldt-jakob disease with protein misfolding cyclic anplification

2017 Fiscal Year Final Research Report

• Project/Area Number: 15K09307
• Research Category: Grant-in-Aid for Scientific Research (C)
• Allocation Type: Multi-year Fund
• Section: 一般
• Research Field: Neurology
• Research Institution: Tohoku University
• Principal Investigator: Takeuchi Atsuko 東北大学, 医学系研究科, 助教 (00535239)
• Co-Investigator(Renkei-kenkyūsha): Kitamoto Tetsuyuki 東北大学, 医学系研究科, 教授 (20192560)
• Project Period (FY): 2015-04-01 – 2018-03-31
• Keywords: プリオン / クロイツフェルト・ヤコブ病 / 試験管内増幅 / 獲得型CJD

Outline of Final Research Achievements

Plaque-type dura mater graft-associated Creutzfeldt-Jacob disease (p-dCJD) prions showed distinct amplification features from those of non p-dCJD (np-dCJD) prions in protein misfolding cyclic amplification (PMCA). Although no amplification from np-dCJD prions was observed with 129M or 129V substrates, p-dCJD prions were amplified drastically with the 129V substrates but not with the 129M substrates despite the PRNP codon 129 incompatibility between seed and substrate. Moreover, we found that type 2 PMCA products were newly generated from p-dCJD prions using type 2 PrPSc-specific antibody, while PrPSc in p-dCJD cases did not react with the type 2 PrPSc-specific antibody. These findings suggest that our PMCA is a useful tool for easily and rapidly identifying acquired CJD associated with the transmission of the V2 CJD strain of codon 129 methionine homozygotes, based on the preference for the 129 V substrate and the type of the amplified products.

Free Research Field

プリオン