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

Author

Listed:
  • M. E. Bruce

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • R. G. Will

    (National CJD Surveillance Unit, Western General Hospital)

  • J. W. Ironside

    (National CJD Surveillance Unit, Western General Hospital)

  • I. McConnell

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • D. Drummond

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • A. Suttie

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • L. McCardle

    (National CJD Surveillance Unit, Western General Hospital)

  • A. Chree

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • J. Hope

    (Institute for Animal Health, Compton)

  • C. Birkett

    (Institute for Animal Health, Compton)

  • S. Cousens

    (London School of Hygiene and Tropical Medicine)

  • H. Fraser

    (Institute for Animal Health, BBSRC/MRC Neuropathogenesis Unit)

  • C. J. Bostock

    (Institute for Animal Health, Compton)

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.

Suggested Citation

  • M. E. Bruce & R. G. Will & J. W. Ironside & I. McConnell & D. Drummond & A. Suttie & L. McCardle & A. Chree & J. Hope & C. Birkett & S. Cousens & H. Fraser & C. J. Bostock, 1997. "Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent," Nature, Nature, vol. 389(6650), pages 498-501, October.
    • Handle: RePEc:nat:nature:v:389:y:1997:i:6650:d:10.1038_39057
    DOI: 10.1038/39057
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    Citations

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    Cited by:

    1. M D Stevenson & J E Oakley & S E Chick & K Chalkidou, 2009. "The cost-effectiveness of surgical instrument management policies to reduce the risk of vCJD transmission to humans," Journal of the Operational Research Society, Palgrave Macmillan;The OR Society, vol. 60(4), pages 506-518, April.
    2. Eric P. M. Grist, 2005. "Transmissible Spongiform Encephalopathy Risk Assessment: The UK experience," Risk Analysis, John Wiley & Sons, vol. 25(3), pages 519-532, June.
    3. Pascual Sanchez-Juan & Matthew T Bishop & Gabor G Kovacs & Miguel Calero & Yurii S Aulchenko & Anna Ladogana & Alison Boyd & Victoria Lewis & Claudia Ponto & Olga Calero & Anna Poleggi & Ángel Carrace, 2015. "A Genome Wide Association Study Links Glutamate Receptor Pathway to Sporadic Creutzfeldt-Jakob Disease Risk," PLOS ONE, Public Library of Science, vol. 10(4), pages 1-14, April.
    4. Gillian McGovern & Stuart Martin & Martin Jeffrey & Glenda Dexter & Steve A C Hawkins & Sue J Bellworthy & Lisa Thurston & Lynne Algar & Lorenzo González, 2016. "Minimum Effective Dose of Cattle and Sheep BSE for Oral Sheep Infection," PLOS ONE, Public Library of Science, vol. 11(3), pages 1-11, March.
    5. Lisa Boden & Ian Handel & Neil Hawkins & Fiona Houston & Helen Fryer & Rowland Kao, 2012. "An Economic Evaluation of Preclinical Testing Strategies Compared to the Compulsory Scrapie Flock Scheme in the Control of Classical Scrapie," PLOS ONE, Public Library of Science, vol. 7(3), pages 1-8, March.

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