Sensitive prion detection by in vitro amplification of abnormal prion protein

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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.