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Absence of protease‐resistant prion protein in the cerebrospinal fluid of Creutzfeldt–Jakob disease
Author(s) -
Wong BoonSeng,
Green Alison J. E.,
Li Ruliang,
Xie Zhiliang,
Pan Tao,
Liu Tong,
Chen Shu G.,
Gambetti Pierluigi,
Sy ManSun
Publication year - 2001
Publication title -
the journal of pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.964
H-Index - 184
eISSN - 1096-9896
pISSN - 0022-3417
DOI - 10.1002/path.872
Subject(s) - cerebrospinal fluid , disease , medicine , protease , pathology , creutzfeldt jakob syndrome , ataxia , scrapie , dementia , prion protein , virology , immunology , biology , enzyme , biochemistry , psychiatry
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 fail to demonstrate any correlation between the presence of protease‐resistant PrP isoform (PrP Sc ) in the CSF and disease manifestation. Copyright © 2001 John Wiley & Sons, Ltd.