Validation of α-Synuclein as a CSF Biomarker for Sporadic Creutzfeldt-Jakob Disease
| Autor: | André Karch, Beata Sikorska, Nadine Gotzmann, Pawel P. Liberski, Inga Zerr, Tobias Knipper, Brit Mollenhauer, Niels Kruse, Franc Llorens, Ting Sun, Saima Zafar, Ewa Golanska, Raquel Sánchez-Valle, Matthias Schmitz, Maria Cramm, Silja Köchy, Andre Fischer |
|---|---|
| Jazyk: | angličtina |
| Předmět: |
Male
0301 basic medicine medicine.medical_specialty Pathology Neurology animal diseases Neuroscience (miscellaneous) diagnosis [Creutzfeldt-Jakob Syndrome] Disease Sporadic Creutzfeldt-Jakob Article Creutzfeldt-Jakob Syndrome Cohort Studies 03 medical and health sciences chemistry.chemical_compound Cellular and Molecular Neuroscience 0302 clinical medicine Cerebrospinal fluid ddc:570 medicine Humans Aged α-Synuclein Electrochemiluminescence Alpha-synuclein business.industry Sporadic Creutzfeldt-Jakob disease cerebrospinal fluid [Creutzfeldt-Jakob Syndrome] Middle Aged 3. Good health nervous system diseases 030104 developmental biology cerebrospinal fluid [Biomarkers] chemistry Csf biomarkers alpha-Synuclein ELISA Female α synuclein Differential diagnosis business Biomarkers 030217 neurology & neurosurgery cerebrospinal fluid [alpha-Synuclein] |
| Zdroj: | Molecular Neurobiology Molecular neurobiology 55(3), 2249-2257 (2018). doi:10.1007/s12035-017-0479-5 |
| ISSN: | 0893-7648 |
| DOI: | 10.1007/s12035-017-0479-5 |
| Popis: | The analysis of cerebrospinal fluid (CSF) biomarkers gains importance in the differential diagnosis of prion diseases. However, no single diagnostic tool or combination of them can unequivocally confirm prion disease diagnosis. Electrochemiluminescence (ECL)-based immunoassays have demonstrated to achieve high diagnostic accuracy in a variety of sample types due to their high sensitivity and dynamic range. Quantification of CSF α-synuclein (a-syn) by an in-house ECL-based ELISA assay has been recently reported as an excellent approach for the diagnosis of sporadic Creutzfeldt-Jakob disease (sCJD), the most prevalent form of human prion disease. In the present study, we validated a commercially available ECL-based a-syn ELISA platform as a diagnostic test for correct classification of sCJD cases. CSF a-syn was analysed in 203 sCJD cases with definite diagnosis and in 445 non-CJD cases. We investigated reproducibility and stability of CSF a-syn and made recommendations for its analysis in the sCJD diagnostic workup. A sensitivity of 98% and a specificity of 97% were achieved when using an optimal cut-off of 820 pg/mL a-syn. Moreover, we were able to show a negative correlation between a-syn levels and disease duration suggesting that CSF a-syn may be a good prognostic marker for sCJD patients. The present study validates the use of a-syn as a CSF biomarker of sCJD and establishes the clinical and pre-analytical parameters for its use in differential diagnosis in clinical routine. Additionally, the current test presents some advantages compared to other diagnostic approaches: it is fast, economic, requires minimal amount of CSF and a-syn levels are stable along disease progression. Electronic supplementary material The online version of this article (doi:10.1007/s12035-017-0479-5) contains supplementary material, which is available to authorized users. |
| Databáze: | OpenAIRE |
| Externí odkaz: |
|