ERA BSE – ERA BSE

Classical bovine spongiform encephalopathy (C-BSE) emerged as a novel prion disease in the 1980s, with huge impacts on UK and European
livestock industries. C-BSE has been effectively controlled by comprehensive bans on feeding animal-derived protein to livestock; however
ongoing surveillance continues to identify occasional cases of C-BSE, as well as atypical forms (L-BSE, H-BSE), which are thought to have a
spontaneous rather than infectious aetiology. C-BSE remains the only zoonotic prion disease identified to date, and eradication is desirable
to avoid re-emergence of a new epidemic in livestock associated with a profound public health risk. C-BSE cases born after the most
stringent feed bans are referred to as “Born After Reinforced Ban” (BARB) cases, and there is a high degree of uncertainty regarding their
origins. Also, there is mounting experimental evidence that C-BSE may exist as a minor hidden fraction of atypical scrapie and atypical BSE
isolates, suggesting possible aetiological links between these diseases and C-BSE. In face of these challenges to the feasibility of complete
BSE eradication in cattle, this project will aim to provide greater understanding of factors that contribute to the persistence of C-BSE, and
develop improved techniques for detection and management of on-going risks. Specifically, we will focus on the role of genetic factors, the
presence of hidden amounts of C-BSE in atypical prion isolates, and environmental contamination/decontamination. The outcomes of the
project will provide tools that can be applied in surveillance, epidemiological investigation and control of future cases of C-BSE and other
animal prion diseases.