BSE to vCJD: do sheep and goats have a part to play?

Christopher Plinston, Rona Wilson, Nora Hunter, Wilfred Goldmann, Rona Barron

Research output: Contribution to conferencePoster

Abstract

In 1996 a new variant form of Creutzfeldt Jacob Disease (vCJD) was reported in humans. vCJD presented with unusual pathology and phenotype compared to those of other human prion diseases such as sporadic CJD (sCJD). Subsequent strain typing experiments demonstrated that vCJD was caused by the same prion agent strain as bovine spongiform encephalopathy (BSE) in cattle. It is widely agreed that exposure to contaminated cattle food products resulted in the transmission of BSE to humans. However, the transmission of cattle BSE to transgenic mouse models expressing human PrP has been inefficient, indicating the presence of a significant transmission barrier between cattle and humans.

Is there another route of infection?

During the BSE epizootic sheep and goats would have undoubtedly been exposed to the same contaminated feed which infected cattle. Sheep and goats can be experimentally infected with BSE via many different routes including oral ingestion proving that the species are susceptible to infection with the BSE agent. To address the transmissibility of sheep- and goat-derived BSE to humans, we performed inoculations into a panel of gene-targeted transgenic mice expressing human PrP. Three lines of transgenic mice were used, representing the genetic diversity in the human population due to the PrP codon 129-methionine/valine polymorphism: HuMM (40%), HuMV (50%), and HuVV (10%). Although previous experiments showed no disease transmission from cattle BSE to any of these human transgenic mouse lines, we show here that experimental sheep- and goat BSE produced pathological evidence of disease transmission in 70% and 41% (respectively) of HuMM transgenic mice. This suggests that sheep- and goat BSE are a greater risk to humans than cattle BSE.

To date there have, however, been only 2 documented cases of natural BSE in goats. Although no evidence of BSE infection has been identified in the field for sheep, the cases of goat BSE show that the natural infection of small ruminants has occurred and highlights the requirement to monitor and prevent BSE from becoming established in small ruminants.
Original languageEnglish
Publication statusPublished - 19 Jun 2013
EventNational Institutes of Bioscience Conference 2013 - The Roslin Institute, Edinburgh, United Kingdom
Duration: 18 Jun 201320 Jun 2013

Conference

ConferenceNational Institutes of Bioscience Conference 2013
CountryUnited Kingdom
CityEdinburgh
Period18/06/1320/06/13

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