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Goat BSE: Improvement of discriminative diagnosis and assessment of infectivity in goat milk and meat - SE1442

Transmissible spongiform encephalopathies (TSEs) are fatal, neurodegenerative diseases of ruminants and man. Sheep and goats can develop natural scrapie with age of onset around 4-5 years, they can also be infected experimentally with BSE and scrapie. Only for goats is there sufficient evidence to suggest that they have succumbed to natural BSE exposure. Although these goat BSE cases are as yet very rare, they have highlighted the need to investigate the risk of humans to consumption of goat products and the risk of animal to animal transmission.
From sheep scrapie research it is known that the genetics of the prion (PrP) protein are crucial in controlling susceptibiity. There is preliminary data to support the notion that PrP genetics also apply in goat TSEs. This proposal will provide experimental evidence for the involvement of the PrP genetics in disease development and the distribution of abnormal prion protein in the affected animal during pathogenesis. These data will be used to assess the human exposure risk posed by goat BSE.
This proposal will also establish the frequencies of allelic variants of the PrP gene in the UK goat population by analysing DNA samples from a minimum of 1000 goats. Samples from animals diagnosed as positive for TSEs in active or passive scrapie surveillance programs in the UK will be included in the genetic tests. This genetic research will help to establish an association between PrP genetics and disease susceptibility. With this knowledge it will be possible to plan genetic breeding programs for the prevention and eradiction of TSEs in goats similar to breeding programs currently performed for sheep scrapie.
The overall strategic objective of this project is to generate data and material on goat BSE that will allow in the greater context of the EU consortium the quantitative assessment of the risk of human exposure to the BSE agent via goats. The aims of this proposal are (1) to establish the PrPSc tissue distribution in BSE affected goats of various PrP genotypes, (2) to investigate the modulation of disease by PrP genetics and (3) to survey the genotypes in the UK goat population. The proposal has been designed to complement the work to be conducted under the EU consortium and will link extensively with the relevant Work Packages of the EU consortium proposal
The Objectives of this proposal:
Task 1: PrP Gene and genetic association with TSE.
1. To define caprine PrP gene haplotypes and their frequencies in UK goat herds.
2. To establish PrP genotype frequencies in UK goat herds.
3. To define the influence of goat PrP genotype on the susceptibility to scrapie in the UK, by analysis of samples from the active and passive surveillance programmes and archival material.
Task 2: BSE dynamics in orally exposed goats.
5 To recover tissue samples from goats orally infected with
6 To study the pathogenesis of first and second passage BSE in orally exposed goats
7 To investigate the effect of PrP genotypes on susceptibility and pathogenesis of BSE in goat.
Project Documents
• FRP - Final Report : Final Report SE1442   (1780k)
Time-Scale and Cost
From: 2006

To: 2012

Cost: £650,403
Contractor / Funded Organisations
Institute for Animal Health (BBSRC), Roslin Institute, Edinburgh (BBSRC)
Animal Health              
Plants and Animals              
Fields of Study
Animal Health