Real-Time Quaking-Induced Conversion Analysis for the Diagnosis of Sporadic Creutzfeldt-Jakob Disease in Korea.
10.3988/jcn.2016.12.1.101
- Author:
Jeong Ho PARK
1
;
Yeong Gon CHOI
;
Yun Jung LEE
;
Seok Joo PARK
;
Hong Seok CHOI
;
Kyung Chan CHOI
;
Eun Kyoung CHOI
;
Yong Sun KIM
Author Information
1. Korea CJD Diagnostic Center, Hallym University, Anyang, Korea. yskim@hallym.ac.kr
- Publication Type:In Vitro ; Original Article
- Keywords:
Creutzfeldt-Jakob disease;
cerebrospinal fluid;
RT-QuIC;
14-3-3;
total tau protein
- MeSH:
14-3-3 Proteins;
Animals;
Cerebrospinal Fluid;
Complement System Proteins;
Creutzfeldt-Jakob Syndrome*;
Diagnosis*;
Humans;
Korea*;
Prion Diseases;
Sensitivity and Specificity;
tau Proteins
- From:Journal of Clinical Neurology
2016;12(1):101-106
- CountryRepublic of Korea
- Language:English
-
Abstract:
BACKGROUND AND PURPOSE: 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.
