Sporadic Creutzfeldt-Jakob disease diagnostic accuracy is improved by a new CSF ELISA 14-3-3γ assay

Neuroscience. 2016 May 13:322:398-407. doi: 10.1016/j.neuroscience.2016.02.057. Epub 2016 Mar 3.

Abstract

Protein 14-3-3 is a reliable marker of rapid neuronal damage, specifically increased in cerebrospinal fluid (CSF) of sporadic Creutzfeldt-Jakob disease (sCJD) patients. Its detection is usually performed by Western Blot (WB), prone to methodological issues. Our aim was to evaluate the diagnostic performance of a recently developed quantitative enzyme-linked immunosorbent (ELISA) assay for 14-3-3γ, in comparison with WB and other neurodegeneration markers. CSF samples from 145 patients with suspicion of prion disease, later classified as definite sCJD (n=72) or Non-prion diseases (Non-CJD; n=73) comprised our population. 14-3-3 protein was determined by WB and ELISA. Total Tau (t-Tau) and phosphorylated Tau (p-Tau) were also evaluated. Apolipoprotein E gene (ApoE) and prionic protein gene (PRNP) genotyping was assessed. ELISA 14-3-3γ levels were significantly increased in sCJD compared to Non-CJD patients (p<0.001), showing very good accuracy (AUC=0.982; sensitivity=97%; specificity=94%), and matching WB results in 81% of all cases. It strongly correlated with t-Tau and p-Tau (p<0.0001), showing slightly higher specificity (14-3-3 WB - 63%; Tau - 90%; p-Tau/t-Tau ratio - 88%). From WB inconclusive results (n=44), ELISA 14-3-3γ correctly classified 41 patients. Additionally, logistic regression analysis selected ELISA 14-3-3γ as the best single predictive marker for sCJD (overall accuracy=93%). ApoE and PRNP genotypes did not influence ELISA 14-3-3γ levels. Despite specificity for 14-3-3γ isoform, ELISA results not only match WB evaluation but also help discrimination of inconclusive results. Our results therefore reinforce this assay as a single screening test, allowing higher sample throughput and unequivocal results.

Keywords: 14-3-3 protein; CSF; ELISA; Tau proteins; Western Blot; sCJD.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 14-3-3 Proteins / cerebrospinal fluid*
  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Apolipoproteins E / genetics
  • Biomarkers / cerebrospinal fluid
  • Blotting, Western
  • Child
  • Creutzfeldt-Jakob Syndrome / cerebrospinal fluid*
  • Creutzfeldt-Jakob Syndrome / genetics
  • Enzyme-Linked Immunosorbent Assay / methods*
  • Female
  • Humans
  • Male
  • Middle Aged
  • Phosphorylation
  • Prion Proteins / genetics
  • Sensitivity and Specificity
  • Young Adult
  • tau Proteins / cerebrospinal fluid

Substances

  • 14-3-3 Proteins
  • Apolipoproteins E
  • Biomarkers
  • MAPT protein, human
  • PRNP protein, human
  • Prion Proteins
  • tau Proteins

Supplementary concepts

  • Creutzfeldt-Jakob Disease, Sporadic