期刊
MOLECULAR NEUROBIOLOGY
卷 55, 期 11, 页码 8586-8591出版社
SPRINGER
DOI: 10.1007/s12035-018-1014-z
关键词
Scrapie; Cerebrospinal fluid; Prion disease; Biomarkers; Prion protein; 14-3-3 protein; Tau protein
资金
- Robert Koch Institute from the Federal Ministry of Health of Germany [1369-341]
- DZNE
- Spanish Ministry of Health, Instituto Carlos III/Fondo Social Europeo [CP16/00041]
- Red Nacional de priones [AGL2015-71764-REDT-MINECO]
- Spanish Ministry of Economy [AGL2015-65560-R]
- Spanish Ministry of Education and Science [FPU14/04348]
- Fondo Europeo de Desarrollo Regional (FEDER) through the Interreg V-A Espana-Francia-Andorra
- Dorothea Schlozer Scholarship (Georg August University - Gottingen)
The analysis of the cerebrospinal fluid (CSF) biomarkers in patients with suspected prion diseases became a useful tool in diagnostic routine. Prion diseases can only be identified at clinical stages when the disease already spread throughout the brain and massive neuronal damage occurs. Consequently, the accuracy of CSF tests detecting non-symptomatic patients is unknown. Here, we aimed to investigate the usefulness of CSF-based diagnostic tests in pre-clinical and clinical naturally occurring scrapie. While decreased total prion protein (PrP) levels and positive PrP seeding activity were already detectable at pre-symptomatic stages, the surrogate markers of neuronal damage total tau (tau) and 14-3-3 proteins were exclusively increased at clinical stages. The present findings confirm that alterations in PrP levels and conformation are primary events in the pathology of prion diseases preceding neuronal damage. Our work also supports the potential use of these tests in the screening of pre-symptomatic scrapie and human prion disease cases.
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