In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc

M. Jones; A.H. Peden; C.V. Prowse; A. Gröner; J.C. Manson; M.L. Turner; J.W. Ironside; I.R. MacGregor; M.W. Head

doi:10.1002/path.2204

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In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc

Journal article

Peer reviewed

In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc

M. Jones, A.H. Peden, C.V. Prowse, A. Gröner, J.C. Manson, M.L. Turner, J.W. Ironside, I.R. MacGregor and M.W. Head

The Journal of Pathology, Vol.213(1), pp.21-26

2007

DOI: https://doi.org/10.1002/path.2204

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Abstract

Variant Creutzfeldt–Jakob disease (vCJD) poses a serious risk of secondary transmission and the need to detect infectivity in asymptomatic individuals is therefore of major importance. Following infection, it is assumed that minute amounts of disease‐associated prion protein (PrPSc) replicate by conversion of the host cellular prion protein (PrPC). Therefore, methods of rapidly reproducing this conversion process in vitro would be valuable tools in the development of such tests. We show that one such technique, protein misfolding cyclic amplification (PMCA), can amplify vCJD PrPSc from human brain tissue, and that the degree of amplification is dependent upon the substrate PRNP codon 129 polymorphism. Both human platelets and transgenic mouse brain are shown to be suitable alternative substrate sources, and amplified PrPSc can be detected using a conformation‐dependent immunoassay (CDI), allowing the detection of putative proteinase K sensitive forms of PrPSc.

Details

Title: In vitro amplification and detection of variant Creutzfeldt-Jakob disease PrPSc
Authors/Creators: M. Jones (Author/Creator) - Western General Hospital
A.H. Peden (Author/Creator) - Western General Hospital
C.V. Prowse (Author/Creator) - Scottish National Blood Transfusion Service
A. Gröner (Author/Creator) - CSL Behring, Emil‐von‐Behring‐Strasse 76, 35041 Marburg, Germany
J.C. Manson (Author/Creator) - Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh EH9 3JF, UK
M.L. Turner (Author/Creator) - Edinburgh Royal Infirmary
J.W. Ironside (Author/Creator) - Western General Hospital
I.R. MacGregor (Author/Creator) - Scottish National Blood Transfusion Service
M.W. Head (Author/Creator) - Western General Hospital
Publication Details: The Journal of Pathology, Vol.213(1), pp.21-26
Publisher: John Wiley & Sons, Ltd.
Identifiers: 991005542277807891
Copyright: Copyright © 2007 Pathological Society of Great Britain and Ireland.
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Collaboration types: Industry collaboration; Domestic collaboration; International collaboration
Citation topics: 1 Clinical & Life Sciences; 1.52 Neurodegenerative Diseases; 1.52.992 Prion Pathogenesis
Web Of Science research areas: Oncology; Pathology
ESI research areas: Clinical Medicine