Abstract
During the past two years, more than 1,000 cases of a neurological disorder of cattle, bovine spongiform encephalopathy (BSE)1, have been confirmed from farms throughout Great Britain. The neurological signs and brain pathology of BSE resemble those produced in other species by the pathogens of scrapie and related disorders. The discovery of fibrils similar to scrapie-associated fibrils in detergent extracts of BSE-affected brain supported the clinical and pathological diagnosis of the disease1, but has been controversial. Scrapie-associated fibrils are found in brain extracts of all species affected by scrapie and diseases caused by related pathogens2,3. They are pathological aggregates of a neuronal membrane protein termed PrP4–6 and a protease-resistant form of PrP7–9 is a molecular marker of scrapie-associated fibrils. In this report, we show the major protein of BSE fibrils is the bovine homologue of PrP as judged by its size, protease resistance, immunoreactivity, lectin binding and partial N-terminal protein sequence. This confirms that BSE is a scrapie-like disease.
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Hope, J., Reekie, L., Hunter, N. et al. Fibrils from brains of cows with new cattle disease contain scrapie-associated protein. Nature 336, 390–392 (1988). https://doi.org/10.1038/336390a0
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DOI: https://doi.org/10.1038/336390a0
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