Human susceptibility to atypical scrapie

C. Plinston, N. Hunter, R.M. Barron

Research output: Contribution to conferenceAbstract


Background: Isolates of classical sheep scrapie are thought to
pose little risk to humans as there have been no documented
links between presence of sheep scrapie and the development
of human TSE disease. However, the link between BSE and the
development of vCJD in humans proves that a risk does exist
from ruminant TSE disease, and therefore all new ruminant
TSEs may potentially be transmissible to humans. Due to increased sensitivity of TSE diagnostic assay systems, a new TSE
of sheep termed ‘atypical scrapie’ has been identified. This disease has been difficult to identify, and is found mainly in
sheep which are previously thought to have a genetic makeup that made them resistant to scrapie. It is unclear whether
this is a new TSE of sheep, an old disease which has only been
identified through increased surveillance, or if it represents
the phenotype of classical scrapie in so called ‘resistant’ sheep
PrP genotypes.
Objectives: The objective of the study is to assess relative
transmissibility of atypical scrapie isolates to humans and the
associated risk to the population.
Methods: In order to determine whether atypical scrapie poses a risk to human health we have transmitted isolates from
three different sheep PrP genotypes to our gene targeted
transgenic mice which express human PrP with the M129V
polymorphism known to be important in human susceptibility to disease. Mice of all three PrP genotypes have been inoculated intracerebrally with atypical scrapie isolates.
Discussion: In order to prevent the emergence of a new human TSE, we need to be able to assess the risk to humans
from new emerging TSEs in livestock. The study of atypical
scrapie infection in these transgenic lines could therefore provide important information on the host range and disease
characteristics associated with such isolates. Preventative
measures could then be put in place before this disease gives
rise to another human disease variant and an underlying level of infection in the population.
Original languageEnglish
Number of pages1
Publication statusPublished - 2009
EventPrion 2009 - Chalkidiki, Greece
Duration: 23 Sep 200925 Sep 2009


ConferencePrion 2009


  • atypical scrapie human M129V prion TSE


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