Prions Research Group

Key aims and expertise

Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurological disorders with long incubation periods (3-6 years in cattle, 15-50 years in humans). The onset of clinical signs of disease becomes evident only during the last months before death. TSEs include scrapie in sheep and goats, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in humans. There remain considerable challenges in the diagnosis and understanding of animal TSEs that we are actively investigating. These include:

• Elucidation of the routes of scrapie transmission and the environmental fate of the TSE agent
• Strain typing to study emerging strains
• Pre-mortem diagnosis

Current projects

Some of our currently active projects are listed below with the name of a contact person for further information:

1. Development of in vitro prion replication assays for the detection of BSE in sheep (Dr Kevin Gough)
2. The transmission and eradication of scrapie prions within an indoor farm environment (Dr Kevin Gough)
3. The replacement of rodent bioassays with in vitro replication assays to measure prion infectivity (Dr Kevin Gough) 

Significant results

Significant highlights of our ongoing research are:

• The demonstration of prion secretion in sheep in a variety of matrices including milk (Maddison et al. (2009) J. Virol. 83:8293-8296), saliva (Maddison et al. (2010) J. Inf. Dis. 201:1672-1676.) and faeces (Terry et al. (2011) Vet. Res. 42:65). Excretion/secretion occurs through long asymptomatic periods of disease development and from sheep with a range of PRNP genotypes, including those with limited lymphoreticular involvement in prion replication (Gough et al. (2011) J. Virol. 86:566-571).
• In addition we have shown that prions are found in a wide range of farm locations including metal, wood, concrete and plastic surfaces both for indoor and outdoors environments (Maddison  et al. (2010) J. Virol. 84:11560-11562); and such infectivity is maintained over years.
• Together, our data reveal the transmission routes of scrapie prions in a natural host and emphasise the significant challenges in controlling the spread of such diseases.