Skip to main content

Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

  • News & Views
  • Published:

Spongiform encephalopathies

The prion's perplexing persistence

Nature volume 392, pages 763–764 (1998)Cite this article

Since prion diseases were identified, we have come to consider such infections as an irrevocable death sentence preceded by severe clinical disease. Take, for example, the cases of iatrogenic Creutzfeldt-Jakob disease elicited by minute traces of the infectious agent, administered through contaminated surgical instruments, hormone preparations or tissue transplants1. On page 770 of this issue, however, Race and Chesebro2 report that infectivity can persist long-term in brain and spleen tissue, without causing the development of clinical symptoms.

There were reasons to believe that exposure to infectious prions may not necessarily lead to clinical disease. For example, mice lacking Prnp (the gene that encodes the normal prion protein, PrPC) are completely immune to clinical disease. They cannot replicate prions, and the traces of infectivity that were occasionally found in the brain several months after injection3,4 have been attributed to persistence in the absence of replication4. Moreover, mice that contain half the normal level of PrPC take a very long time to develop symptoms after intracerebral inoculation5. Finally, if immuno-deficient mice are inoculated peripherally with the scrapie agent, they do not fall ill, despite the occasional presence of large prion loads in their brains6.

This is a preview of subscription content, access via your institution

Access options

Buy this article

  • Purchase on Springer Link
  • Instant access to full article PDF
Buy now

Prices may be subject to local taxes which are calculated during checkout

Figure 1: Due to the species barrier between mice and hamsters, prions originating from one species produce spongiform encephalopathy rather quickly when transferred to a further animal of the same species, yet only produce disease — if any — with much longer latency when passed to the other species.

References

  1. Brown, P., Preece, M. A. & Will, R. G. Lancet 340, 24–27 (1992).

    Google Scholar 

  2. Race, R. & Chesebro, B. Nature 392, 770 (1998).

    Article  ADS  CAS  PubMed  Google Scholar 

  3. Sakaguchi, S.et al. J. Virol. 69, 7586–7592 (1995).

    Google Scholar 

  4. Sailer, A., Büeler, H., Fischer, M., Aguzzi, A. & Weissmann, C. Cell 77, 967–968 (1994).

    Google Scholar 

  5. Büeler, H.et al. Mol. Med. 1, 19–30 (1994).

    Google Scholar 

  6. Klein, M. A.et al. Nature 390, 687–690 (1997).

    Article  ADS  CAS  PubMed  Google Scholar 

  7. Hill, A. F.et al. Nature 389, 448–450 (1997).

    Article  ADS  CAS  PubMed  Google Scholar 

  8. Bruce, M. E.et al. Nature 389, 498–501 (1997).

    Article  ADS  CAS  PubMed  Google Scholar 

  9. Kimberlin, R. H. & Walker, C. A. J. Gen. Virol. 42, 107–117 (1979).

    Google Scholar 

  10. Brandner, S.et al. Proc. Natl Acad. Sci. USA 93, 13148–13151 (1996).

    Google Scholar 

  11. Korth, C.et al. Nature 390, 74–77 (1997).

    Article  ADS  CAS  PubMed  Google Scholar 

  12. Scott, M.et al. Cell 59, 847–857 (1989).

    Google Scholar 

  13. Prusiner, S. B.et al. Cell 63, 673–686 (1990).

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. the Institute of Neuropathology, University Hospital of Zürich, CH-8091, Zürich, Switzerland

    Adriano Aguzzi

  2. the Institute of Molecular Biology, University of Zürich, Hönggerberg, 8093, Zürich, Switzerland

    Charles Weissmann

Authors
  1. Adriano Aguzzi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Charles Weissmann
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Aguzzi, A., Weissmann, C. The prion's perplexing persistence. Nature 392, 763–764 (1998). https://doi.org/10.1038/33812

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1038/33812

This article is cited by

Search

Advanced search

Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing