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Prion diseases are fatal transmissible neurodegenerative diseases that are currently incurable. Although the risk to humans is generally considered low, cases of Creutzfeldt-Jacob in humans are a fact, and the recent discovery of two bovine spongiform encephalopathy (BSE) cases in goats has shown that the species barrier is not watertight. The goatBSE project aims at providing sound scientific information to allow the risk assessment of human exposure to BSE via goat milk, meat and products thereof.




In light of the known ability of the BSE agent to cross the animal/human species barrier, recent evidence establishing the presence of BSE in goat is especially alarming, as it represents a new potential risk of food-born contamination to human consumers of goat milk and meat products.


Aims of the project

The main objective is to determine the tissue distribution of BSE after oral exposure of goats while simultaneously generating indispensable data on genetic susceptibility in most common used production breeds.


The project more specified aims at:

   1. providing data for the evaluation of human risk associated with goat BSE passage in goat,

   2. providing pathogenesis data and biological material from first and second passage BSE in goats,

   3. evaluating the possibility of BSE self-maintenance in goat herds through maternal or horizontal transmission,

   4. validating and improving our ability to detect caprine BSE and discriminate it from scrapie in goats.



The project itself has been designed to address the following specific objectives;

  • To determine the tissue infectivity distribution of BSE after oral exposure of goats. This will involve work focussing on CNS tissues, peripheral nervous tissues, lymph nodes, intestines, muscle tissues and milk.
  • To determine the influence of PrP genotype on susceptibility of goats for TSEs and in particular BSE. This will involve case-control studies in scrapie infected herds, experimental inoculations of goats having different PrP genotypes with different TSE inoculums and work addressing the transmissibility/species barriers by different goats and transgenic caprinised mice inoculations as well as rapid in vitro systems.
  • To generate/collect data and materials that will allow exploitation to quantify the risk posed to humans by the consumption of milk (milk products) and meat of goats.
  • To improve animal models as acceptable diagnostic assays for TSEs (in particular BSE) in small ruminants. This will involve the generation of caprinised transgenic mice carrying the PrP alleles making them highly susceptible to small ruminant TSEs and compare them to the classical mouse bioassays. In addition, goats harbouring the most susceptible PrP alleles will be assessed as a rapid/sensitive model for small ruminant TSEs.
  • To assess currently standard diagnostic methods and optimised methods thereof for the detection and strain differentiation of TSEs in goats.
  • To assess the feasibility of breeding programmes to improve existing prevention and control strategies for TSEs in goat.
  • To determine the geographical mapping of prions in goats. This will involve the collection of a panel of TSE goat cases covering the European geographical zone and select amongst them cases for strain-typing with biochemical methods and biological bioassays.



Expected Results

It is expected that the project will have a number of important outcomes with immediate relevance to the "European" problem of BSE in goats:

  • BSE infectivity distribution in goats and goat products.
  • Improved animal models for detection and discrimination of BSE from other TSE strains (in goat).
  • Improved and validated (existing) diagnostics for goat BSE and scrapie.
  • Additional guidelines to actively control TSEs in goats by breeding or selected culling strategies (also suitable to end-users/producers).
  • TSE in goat reference point for all stakeholders including consumers, producers, scientific community and policy makers.


Potential applications

Our approach will integrate the predicted influence of PrP gene polymorphisms on scrapie and BSE susceptibility so that it could potentially be used for the control of field TSE outbreaks in goats. We will also document European field TSE strain variability in goats by recruiting a large number of TSE goat isolates from affected European countries. Already established or specifically created animal models (strain typing) and biochemical tools (PrPSc typing), will be investigated for their ability to efficiently discriminate goat BSE/scrapie in goats. Finally, by measuring infectivity in various tissues (including skeletal muscle) and secretions (milk), collected from goats at different stages of BSE infection, we will provide indispensable essential valuable data for quantitative risk assessment.

EU Logo GoatBSE is a STREP supported by funding under the 6th Research Framework Programme of the European Union, EU Contract No. FOOD-CT-36353. This website represents the views of the Authors, not the European Commission. The Commission is not liable for any use that may be made of the information.
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