UCL Discovery
UCL home » Library Services » Electronic resources » UCL Discovery

Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein

Joiner, S; Asante, EA; Linehan, JM; Brock, L; Brandner, S; Bellworthy, SJ; Simmons, MM; ... Wadsworth, JDF; + view all (2018) Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein. Journal of the Neurological Sciences , 386 pp. 4-11. 10.1016/j.jns.2017.12.038. Green open access

[img]
Preview
Text
Joiner_Experimental sheep.pdf - Published version

Download (1MB) | Preview

Abstract

The epizootic prion disease of cattle, bovine spongiform encephalopathy (BSE), causes variant Creutzfeldt-Jakob disease (vCJD) in humans following dietary exposure. While it is assumed that all cases of vCJD attributed to a dietary aetiology are related to cattle BSE, sheep and goats are susceptible to experimental oral challenge with cattle BSE prions and farmed animals in the UK were undoubtedly exposed to BSE-contaminated meat and bone meal during the late 1980s and early 1990s. Although no natural field cases of sheep BSE have been identified, it cannot be excluded that some BSE-infected sheep might have entered the European human food chain. Evaluation of the zoonotic potential of sheep BSE prions has been addressed by examining the transmission properties of experimental brain isolates in transgenic mice that express human prion protein, however to-date there have been relatively few studies. Here we report that serial passage of experimental sheep BSE prions in transgenic mice expressing human prion protein with methionine at residue 129 produces the vCJD phenotype that mirrors that seen when the same mice are challenged with vCJD prions from patient brain. These findings are congruent with those reported previously by another laboratory, and thereby strongly reinforce the view that sheep BSE prions could have acted as a causal agent of vCJD within Europe.

Type: Article
Title: Experimental sheep BSE prions generate the vCJD phenotype when serially passaged in transgenic mice expressing human prion protein
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.jns.2017.12.038
Publisher version: https://doi.org/10.1016/j.jns.2017.12.038
Language: English
Additional information: Copyright © 2018 The Authors. This is an open access article under the CC BY license (http://creativecommons.org/licenses/BY/4.0/).
Keywords: Bovine spongiform encephalopathy (BSE), Prions, Prion disease, Variant Creutzfeldt-Jakob disease (vCJD), Sheep-BSE, Transmissible spongiform encephalopathy (TSE)
UCL classification: UCL
UCL > Provost and Vice Provost Offices
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > MRC Prion Unit at UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Institute of Prion Diseases > UCL Institute of Prion Diseases Support
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Brain Sciences > UCL Queen Square Institute of Neurology > Neurodegenerative Diseases
URI: https://discovery.ucl.ac.uk/id/eprint/10041642
Downloads since deposit
49Downloads
Download activity - last month
Download activity - last 12 months
Downloads by country - last 12 months

Archive Staff Only

View Item View Item