1887

Abstract

New variant Creutzfeldt-Jakob disease (vCJD) and bovine spongiform encephalopathy (BSE) are caused by the same strain of pathogen and, as sheep can develop experimental BSE, this has raised concern that humans may be at risk from eating mutton if BSE has naturally transmitted to sheep. Biochemical typing of abnormal prion proteins (PrPsc) has been suggested to detect BSE in sheep. Although this approach is ingenuous, we can now report biochemical evidence of strain variation in contemporary and archival brain tissue from cases of experimental BSE or experimental and natural scrapie in sheep. Interestingly, we found at least one isolate of natural scrapie (CH 1641) with a very similar, but not identical, PrPsc profile to BSE but which differs from BSE in its transmission characteristics to mice.

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1999-01-01
2022-05-28
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References

  1. Bessen R. A., Marsh R. F. 1994; Distinct PrP properties suggest the molecular basis of strain variation in transmissible mink encephalopathy. Journal of Virology 68:7859–7868
    [Google Scholar]
  2. Bruce M., Chree A., McConnell I., Foster J., Pearson G., Fraser H. 1994; Transmission of bovine spongiform encephalopathy and scrapie to mice – strain variation and the species barrier. Philosophical Transactions of the Royal Society of London Series B Biological Sciences 343:405–411
    [Google Scholar]
  3. Bruce M. E., Will R. G., Ironside J. W., McConnell I., Drummond D., Suttie A., McCardle L., Chree A., Hope J., Birkett C., Cousens S., Fraser H., Bostock C. J. 1997; Transmissions to mice indicate that ‘new variant’ CJD is caused by the BSE agent. Nature 389:498–501
    [Google Scholar]
  4. Collinge J., Sidle K. C. L., Meads J., Ironside J., Hill A. F. 1996; Molecular analysis of prion strain variation and the aetiology of new variant CJD. Nature 383:685–690
    [Google Scholar]
  5. Dickinson A. G. 1976; Scrapie in sheep and goats. In Slow Virus Diseases of Animals and Man pp 209–241 Edited by Kimberlin R. H. Amsterdam: North-Holland;
    [Google Scholar]
  6. Dickinson A. G., Outram G. W. 1988; Genetic aspects of unconventional virus infections: the basis of the virino hypothesis. CIBA Foundation Symposium 135:63–83
    [Google Scholar]
  7. Foster J. D., Dickinson A. G. 1988; The unusual properties of CH1641, a sheep-passaged isolate of scrapie. Veterinary Record 123:5–8
    [Google Scholar]
  8. Foster J. D., Hope J., Fraser H. 1993; Transmission of bovine spongiform encephalopathy to sheep and goats. Veterinary Record 133:339–341
    [Google Scholar]
  9. Foster J. D., Bruce M., McConnell I., Chree A., Fraser H. 1996; Detection of BSE infectivity in brain and spleen of experimentally infected sheep. Veterinary Record 138:546–548
    [Google Scholar]
  10. Fraser H., Foster J. D. 1993; Transmission to mice, sheep and goats and bioassay of bovine tissues. In Transmissible Spongiform Encephalopathies pp 145–159 Edited by Bradley R., Marchant B. Brussels, Belgium: Commission of the European Communities;
    [Google Scholar]
  11. Goldmann W., Hunter N., Smith G., Foster J., Hope J. 1994; PrP genotype and agent effects in scrapie: change in allelic interaction with different isolates of agent in sheep, a natural host of scrapie. Journal of General Virology 75:989–995
    [Google Scholar]
  12. Hope J. 1994; The nature of the scrapie agent – the evolution of the virino. Annals of the New York Academy of Sciences 724:282–289
    [Google Scholar]
  13. Hill A. F., Desbruslais M., Joiner S., Sidle K. C. L., Gowland I., Collinge J., Doey L. J., Lantos P. 1997; The same prion strain causes vCJD and BSE. Nature 389:448–450
    [Google Scholar]
  14. Kascsak R. J., Rubenstein R., Merz P. A., Carp R. I., Robakis N. K., Wisniewski H. M., Diringer H. 1986; Immunological comparison of scrapie-associated fibrils isolated from animals infected with four different scrapie strains. Journal of Virology 59:676–683
    [Google Scholar]
  15. Kocisko D. A., Lansbury P. T., Caughey B. 1996; Partial unfolding and refolding of scrapie-associated prion protein: evidence for a critical 16-kDa C-terminal domain. Biochemistry 35:13434–13442
    [Google Scholar]
  16. Moore R. C., Hope J., McBride P. A., McConnell I., Selfridge J., Melton D. W., Manson J. C. 1998; Mice with gene targetted prion protein alterations show that Prnp, Sinc and Prni are congruent. Nature Genetics 18:118–125
    [Google Scholar]
  17. Parchi P., Castellani R., Capellari S., Ghetti B., Young K., Chen S. G., Farlow M., Dickson D. W., Sima A. A. F., Trojanowski J. Q., Petersen R. B., Gambetti P. 1996; Molecular basis of phenotypic variability in sporadic Creutzfeldt–Jakob disease. Annals Of Neurology 39:767–778
    [Google Scholar]
  18. Prusiner S. B. 1991; Molecular biology of prion diseases. Science 252:1515–1522
    [Google Scholar]
  19. Somerville R. A., Chong A., Mulqueen O. U., Birkett C. R., Wood S. C. E. R., Hope J. 1997; Biochemical typing of scrapie strains. Nature 386:564
    [Google Scholar]
  20. Warwicker J. 1997; Species barriers in a model for specific prion protein dimerization. Biochemical and Biophysical Research Communications 232:508–512
    [Google Scholar]
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