Absence of protease-resistant prion protein in the cerebrospinal fluid of Creutzfeldt-Jakob disease

The Journal of Pathology
B S WongM S Sy

Abstract

Creutzfeldt-Jakob disease (CJD), believed to be caused by a protease-resistant isoform of prion protein (PrP(Sc)), usually manifests itself as a clinically distinctive age-related dementia because of its rapid progression, occasionally accompanied by cerebellar ataxia. Recently, a variant CJD (vCJD) has been described, which has prominent early psychiatric symptoms and an earlier age of death. Although cerebrospinal fluid (CSF) is part of the extracellular fluid of the central nervous system (CNS), the bulk of its proteins are derived from the plasma and there is increasing concern about possible transmission of prion disease by blood. As investigation of CSF has played a significant role in the diagnosis and management of several neurological diseases, it was decided to characterize PrP present in the CSF of CJD individuals. Significant variation was observed in the level of PrP in the CSF from both non-CJD and CJD (including vCJD) patients, and the detected PrP forms are protease-sensitive. Using a conformation-dependent immunoassay, it was further demonstrated that the PrP detected in the CSF from CJD patients was broadly similar in conformation to that found in non-CJD patients. Taken together, the results of this study fai...Continue Reading

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Citations

Jul 17, 2003·Journal of Neuroimmunology·Jae-Il KimRichard Rubenstein
Jul 3, 2009·Protein Engineering, Design & Selection : PEDS·Christina D OrrúByron Caughey
May 27, 2008·Veterinary Immunology and Immunopathology·Laura J VellaAndrew F Hill
Mar 27, 2016·Progress in Neurobiology·Franc LlorensInga Zerr
Jun 5, 2004·The World Journal of Biological Psychiatry : the Official Journal of the World Federation of Societies of Biological Psychiatry·Gábor G KovácsHerbert Budka
Nov 14, 2006·The Journal of General Virology·Nicole Picard-HagenPierre-Louis Toutain

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