Sensitive prion detection by in vitro amplification of abnormal prion protein

异常形式朊病毒蛋白（PrPSc）体外扩增技术的发展为开发一种新的朊病毒疾病诊断方法提供了可能。然而，超灵敏的PrPSc检测在容易获得的标本，如脑脊液（CSF）尚未成功的人类朊病毒疾病。在这项研究中，我们开发了一种新的PrPSc扩增检测，指定为“实时QUIC（RT-QUIC）"，允许检测微量的PrPSc稀释散发克罗伊茨费尔特？雅各布病（sCJD）脑匀浆。此外，我们首先在一系列日本受试者中评估了该技术，然后在来自澳大利亚的30份脑脊液标本的盲法研究中评估了该技术，其灵敏度超过80%，特异性超过100%。这些研究结果表明，有前途的增强诊断能力的RT-QUIC在疑似CJD的死前评估。

The development of in vitro amplification technology for abnormal forms of prion protein (PrPSc) has generated the possibility for developing a novel diagnostic test for prion diseases. However, ultrasensitive PrPSc detection in easily accessible specimens such as cerebrospinal fluid (CSF) has not yet been successful in human prion diseases. In this study, we developed a new PrPSc amplification assay, designated "real-time QUIC (RT-QUIC)", allows for the detection of a minute amount of PrPSc in diluted sporadic Creutzfeldt?Jakob disease (sCJD) brain homogenate. Moreover, we assessed the technique first in a series of Japanese subjects and then in a blind study of 30 cerebrospinal fluid specimens from Australia, which achieved greater than 80% sensitivity and 100% specificity. These findings indicate the promising enhanced diagnostic capacity of RT-QUIC in the antemortem evaluation of suspected CJD.