Abstract
Bovine spongiform encephalopathy (BSE) was first recognised in the UK in 1986 as a neurodegenerative disease of cattle similar to scrapie in sheep. Its transmissibility was first demonstrated by the production of neurological disease in mice following inoculation with brain material derived from affected bovine brain. Subsequently the causative agent has been shown to cause disease following experimental transmission to several mammalian species. Since different strains of scrapie agents can be characterised on the basis of the detailed course of the disease they cause following inoculation into genetically defined strains of mice, BSE-affected brain homogenates were inoculated into a panel of mice strains. The BSE agent has been biologically “typed” by characterising disease incubation times and neuropathological lesions following inoculation of a panel of 5 genetically defined strains of mice. The results show that the BSE agent is clearly a “scrapie-like” agent but its detailed biological properties indicate that it is distinguishable from previously known strains and a current isolate of scrapie. The results of these and other experiments on the genetic polymorphisms in the bovine PrP gene conducted within the Institute for Animal Health are presented and discussed.
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