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Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent

Nature volume 389, pages 498–501 (1997)Cite this article

Abstract

There are many strains of the agents that cause transmissible spongiform encephalopathies (TSEs) or ‘prion’ diseases. These strains are distinguishable by their disease characteristics in experimentally infected animals, in particular the incubation periods and neuropathology they produce in panels of inbred mouse strains1,2,3,4. We have shown that the strain of agent from cattle affected by bovine spongiform encephalopathy (BSE) produces a characteristic pattern of disease in mice that is retained after experimental passage through a variety of intermediate species5,6,7. This BSE ‘signature’ has also been identified in transmissions to mice of TSEs of domestic cats and two exotic species of ruminant6,8, providing the first direct evidence for the accidental spread of a TSE between species. Twenty cases of a clinically and pathologically atypical form of Creutzfeldt–Jakob disease (CJD), referred to as ‘new variant’ CJD (vCJD)9, have been recognized in unusually young people in the United Kingdom, and a further case has been reported in France10. This has raised serious concerns that BSE may have spread to humans, putatively by dietary exposure. Here we report the interim results of transmissions of sporadic CJD and vCJD to mice. Our data provide strong evidence that the same agent strain is involved in both BSE and vCJD.

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Figure 1: Incubation times in mice with spongiform encephalopathies.
Figure 2: Lesion profiles for mice following transmission of spongiform encephalopathies.
Figure 3: Survival curves for mice following transmission of spongiform encephalopathies.

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Acknowledgements

We acknowledge the contribution of A. Dickinson, who pioneered TSE strain discrimiantion in the 1960s. We also thank M. Brady, F. Purdie, L. Hunter, K. Lamza, S. Mack and other staff at the Neuropathogenesis Unit for technical support. This work was supported by the Department of Health, MRC, BBSRC and MAFF.

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Authors and Affiliations

  1. Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit, West Mains Road, Edinburgh, EH9 3JF, UK

    M. E. Bruce, I. McConnell, D. Drummond, A. Suttie, A. Chree & H. Fraser

  2. National CJD Surveillance Unit, Western General Hospital, Edinburgh, EH4 2XU, UK

    R. G. Will, J. W. Ironside & L. McCardle

  3. Institute for Animal Health, Compton, Newbury, RG20 7NN, Berkshire, UK

    J. Hope, C. Birkett & C. J. Bostock

  4. Department of Epidemiology and Population Science, London School of Hygiene and Tropical Medicine, London, WC1E 7HT, UK

    S. Cousens

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  1. M. E. Bruce
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  4. I. McConnell
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  8. A. Chree
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  12. H. Fraser
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Correspondence to M. E. Bruce.

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Bruce, M., Will, R., Ironside, J. et al. Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 389, 498–501 (1997). https://doi.org/10.1038/39057

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