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Mol Neurobiol. 2018 Nov;55(11):8586-8591. doi: 10.1007/s12035-018-1014-z. Epub 2018 Mar 23.

Cerebrospinal Fluid Prion Disease Biomarkers in Pre-clinical and Clinical Naturally Occurring Scrapie.

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CIBERNED (Network Centre for Biomedical Research of Neurodegenerative Diseases), Institute Carlos III, Ministry of Health, L'Hospitalet de Llobregat, Feixa Llarga s/n, 08907, Barcelona, Spain.
IDIBELL, Bellvitge Biomedical Research Institute L'Hospitalet de Llobregat, Barcelona, Spain.
Department of Neurology, University Medical Center, Göttingen, Germany.
Centro de Encefalopatías y Enfermedades Transmisibles Emergentes (CEETE), Veterinary Faculty, Instituto Agroalimentario de Aragón- IA2, Universidad de Zaragoza-CITA, Zaragoza, Spain.
Instituto de Investigación Sanitaria de Aragón (IIS), Zaragoza, Spain.
Department of Neurology, University Medical Center, Göttingen, Germany.
German Centre for Neurodegenerative Diseases (DZNE), Göttingen, Germany.
AJ Roboscreen GmbH, Leipzig, Germany.


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.


14-3-3 protein; Biomarkers; Cerebrospinal fluid; Prion disease; Prion protein; Scrapie; Tau protein

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