Progress in understanding the epidemiology and pathogenesis of TSEs and in assessment of risk of various products for humans has been hampered by the lack of a convenient assay for the infectious agents. Transgenic mice expressing the """"""""prion"""""""" protein (PrP) genes of other species have been derived and proposed as improved assay animals for the agents of TSEs. We propose to assemble a Panel of TSE Reference Agents including field specimens of bovine spongiform encephalopathy, American scrapie of sheep and goats, chronic wasting disease of American deer and elk, mink encephalopathy and the human TSEs (Creutzfeldt-Jakob disease and Gerstmann-Straussler syndrome) to test directly the sensitivity of candidate lines of transgenic mice to detect those agents.
