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Fibrils from brains of cows with new cattle disease contain scrapie-associated protein

Nature volume 336, pages 390–392 (1988)Cite this article

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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Authors and Affiliations

  1. Institute for Animal Health, AFRC & MRC Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh, EH9 3JF, UK

    James Hope, Laura J. D. Reekie & Nora Hunter

  2. Zentrum für Molekulare Biologic, Universität Heidelberg, Im Neuenheimer Feld 282, D-6000, Heidelberg, FRG

    Gerd Multhaup & Konrad Beyreuther

  3. Ministry of Agriculture, Fisheries and Food, Central Veterinary Laboratory, New Haw, Weybridge, Surrey, KT15 3NB, UK

    Heather White, Anthony C. Scott, Michael J. Stack, Michael Dawson & Gerald A. H. Wells

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  1. James Hope
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  9. Michael Dawson
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  10. Gerald A. H. Wells
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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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