The shifting biology of prions

Brain Res Brain Res Rev. 2001 Oct;36(2-3):241-8. doi: 10.1016/s0165-0173(01)00100-x.


Transmissible spongiform encephalopathies (TSEs), or prion diseases, are rare fatal neurodegenerative diseases of humans and animals. Although some TSEs, like scrapie in sheep, have been known to exist for centuries, bovine spongiform encephalopathy (BSE) was recognized only 15 years ago. New variant Creutzfeldt-Jakob disease (nvCJD) of humans is probably caused by consumption of BSE-infected materials. The nature of the infectious agent is not fully elucidated, but substantial evidence suggests that it is devoid of nucleic acids and consists at least in part of an abnormal form of a host protein termed PrP(C). Despite their rarity, prion diseases have become an important topic in public health and basic research because of the connection between nvCJD and BSE and also because of the unusual biological attributes of the infectious agent.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Central Nervous System / immunology*
  • Central Nervous System / pathology
  • Central Nervous System / physiopathology
  • Creutzfeldt-Jakob Syndrome / genetics
  • Creutzfeldt-Jakob Syndrome / pathology
  • Creutzfeldt-Jakob Syndrome / physiopathology
  • Diagnostic Tests, Routine
  • Humans
  • Mice
  • Mice, Transgenic
  • Prion Diseases / genetics*
  • Prion Diseases / pathology
  • Prion Diseases / physiopathology
  • Prions / genetics*
  • Prions / metabolism
  • Prions / pathogenicity
  • Scrapie / genetics
  • Scrapie / pathology
  • Scrapie / physiopathology


  • Prions