Mammalian prion biology: one century of evolving concepts

Cell. 2004 Jan 23;116(2):313-27. doi: 10.1016/s0092-8674(03)01031-6.


Prions have been responsible for an entire century of tragic episodes. Fifty years ago, kuru decimated the population of Papua New Guinea. Then, iatrogenic transmission of prions caused more than 250 cases of Creutzfeldt-Jakob disease. More recently, transmission of bovine spongiform encephalopathy to humans caused a widespread health scare. On the other hand, the biology of prions represents a fascinating and poorly understood phenomenon, which may account for more than just diseases and may represent a fundamental mechanism of crosstalk between proteins. The two decades since Stanley Prusiner's formulation of the protein-only hypothesis have witnessed spectacular advances, and yet some of the most basic questions in prion science have remained unanswered.

Publication types

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

MeSH terms

  • Animals
  • Cattle
  • Creutzfeldt-Jakob Syndrome / etiology
  • Encephalopathy, Bovine Spongiform / etiology
  • Fungal Proteins / physiology
  • Humans
  • Immunoassay / methods
  • Models, Anatomic
  • Models, Biological
  • Prion Diseases / diagnosis
  • Prions / metabolism
  • Prions / physiology*
  • Protein Conformation


  • Fungal Proteins
  • Prions