Altered Prion protein expression pattern in CSF as a biomarker for Creutzfeldt-Jakob disease.

PloS One
Mauricio TorresClaudio Hetz

Abstract

Creutzfeldt-Jakob disease (CJD) is the most frequent human Prion-related disorder (PrD). The detection of 14-3-3 protein in the cerebrospinal fluid (CSF) is used as a molecular diagnostic criterion for patients clinically compatible with CJD. However, there is a pressing need for the identification of new reliable disease biomarkers. The pathological mechanisms leading to accumulation of 14-3-3 protein in CSF are not fully understood, however neuronal loss followed by cell lysis is assumed to cause the increase in 14-3-3 levels, which also occurs in conditions such as brain ischemia. Here we investigated the relation between the levels of 14-3-3 protein, Lactate dehydrogenase (LDH) activity and expression of the prion protein (PrP) in CSF of sporadic and familial CJD cases. Unexpectedly, we found normal levels of LDH activity in CJD cases with moderate levels of 14-3-3 protein. Increased LDH activity was only observed in a percentage of the CSF samples that also exhibited high 14-3-3 levels. Analysis of the PrP expression pattern in CSF revealed a reduction in PrP levels in all CJD cases, as well as marked changes in its glycosylation pattern. PrP present in CSF of CJD cases was sensitive to proteases. The alterations in PrP ex...Continue Reading

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Citations

Apr 26, 2013·Journal of Neuroimmune Pharmacology : the Official Journal of the Society on NeuroImmune Pharmacology·Bezawit MegraJoan W Berman
Mar 27, 2016·Progress in Neurobiology·Franc LlorensInga Zerr
May 23, 2015·The Psychiatric Clinics of North America·Kalyani Kansal, David J Irwin
Mar 21, 2017·Clinica Chimica Acta; International Journal of Clinical Chemistry·Tanushri ChatterjiRaja Roy
Aug 1, 2018·Molecular Neurobiology·Anna Villar-PiquéFranc Llorens
Aug 23, 2016·Annals of Clinical and Translational Neurology·Sanam Soomro, Chandra Mohan
Apr 3, 2019·Proceedings of the National Academy of Sciences of the United States of America·Sonia M VallabhStuart L Schreiber
Dec 17, 2018·Journal of Molecular Neuroscience : MN·Thais Guimarães Martins NeryFabio Passetti
Jul 15, 2015·The Journal of Biological Chemistry·Mauricio TorresClaudio Hetz
Jul 3, 2021·International Journal of Molecular Sciences·Óscar López-PérezInmaculada Martín-Burriel
Jul 7, 2021·Expert Review of Proteomics·Nimisha GuptaSaima Wajid

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Methods Mentioned

BETA
glycosylation
glycosylations

Software Mentioned

GraphPad
SigmaPlot
ImageJ

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