title: Real-Time Quaking-Induced Conversion Analysis for the Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Korea.
abstract: OBJECTIVE
  The level of 14-3-3 protein in the cerebrospinal fluid (CSF) is increased in Creutzfeldt-Jakob disease (CJD) patients, which has led to it being used as a clinical biomarker for the ante-mortem diagnosis of human prion diseases. However, the specificity of the 14-3-3 protein is less reliable for CJD diagnosis. Newly developed assays including real-time quaking-induced conversion (RT-QuIC) have made it possible to detect the PrPSc-like abnormal prion isoform with a high sensitivity in animal and human specimens that might contain a minute amount of PrP(Sc) due to in vitro prion replication.
  
  
  METHODS
  This study applied a highly sensitive RT-QuIC assay using recombinant human PrP to detect PrP(Sc) in the CSF of 81 patients with sporadic CJD (sCJD) in Korea.
  
  
  RESULTS
  RT-QuIC analysis of the CSF samples based on the expression levels of 14-3-3 and total tau proteins revealed positivity in 62 of 81 sCJD patients (sensitivity of 76.5%) but no positive results in the 100 non-CJD patients.
  
  
  CONCLUSIONS
  The sensitivity of the RT-QuIC in this study was similar to that in some previous reports, and the specificity of RT-QuIC was higher than that of 14-3-3 in CSF, suggesting that RT-QuIC analysis can complement the weakness of the specificity of 14-3-3 for the diagnosis of sCJD. These results indicate that RT-QuIC might be very useful for the rapid and specific diagnosis of sCJD and provide a practical novel method for the ante-mortem diagnosis of human prion diseases.
authors.affiliation: Korea CJD Diagnostic Center, Hallym University, Anyang, Korea.|Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea.
authors.forename: Jeong Ho
authors.initials: JH
authors.lastname: Park
authors.affiliation: Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea.
authors.forename: Yeong Gon
authors.initials: YG
authors.lastname: Choi
authors.affiliation: Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea.
authors.forename: Yun Jung
authors.initials: YJ
authors.lastname: Lee
authors.affiliation: Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea.
authors.forename: Seok Joo
authors.initials: SJ
authors.lastname: Park
authors.affiliation: Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea.
authors.forename: Hong Seok
authors.initials: HS
authors.lastname: Choi
authors.affiliation: Department of Pathology, Chuncheon Sacred Heart Hospital, Hallym University College of Medicine, Chuncheon, Korea.|Korea CJD Autopsy Center, Hallym University Sacred Heart Hospital, Anyang, Korea.
authors.forename: Kyung Chan
authors.initials: KC
authors.lastname: Choi
authors.affiliation: Department of Biomedical Gerontology, Graduate School of Hallym University, Chuncheon, Korea.|Laboratory of Cellular Aging and Neurodegeneration, Ilsong Institute of Life Science, Hallym University, Anyang, Korea. ekchoi@hallym.ac.kr.
authors.forename: Eun Kyoung
authors.initials: EK
authors.lastname: Choi
authors.affiliation: Korea CJD Diagnostic Center, Hallym University, Anyang, Korea.|Laboratory of Transmissible Spongiform Encephalopathy, Ilsong Institute of Life Science, Hallym University, Anyang, Korea. yskim@hallym.ac.kr.
authors.forename: Yong Sun
authors.initials: YS
authors.lastname: Kim
keywords: 14-3-3; Creutzfeldt-Jakob disease; RT-QuIC; cerebrospinal fluid; total tau protein