Molecular biology of prions

Charles Weissmann, Masato Enari, Peter Christian Klöhn, Daniela Rossi, Eckhard Flechsig

Research output: Contribution to journalArticlepeer-review

Abstract

The "protein only" hypothesis holds that the infectious agent causing transmissible spongiform encephalopathies is a conformational isomer of PrP, a host protein predominantly expressed in brain and is strongly supported by many lines of evidence. Prion diseases are so far unique among conformational diseases in that they are transmissible, not only experimentally but also by natural routes, mainly by ingestion. The pathway of prions to the brain has been elucidated in outline. A striking feature of prions is their extraordinary resistance to conventional sterilisation procedures, and their capacity to bind to surfaces of metal and plastic without losing infectivity. This property, first observed in a clinical setting, is now being investigated in experimental settings, both in animals and in cell culture.

Original languageEnglish
Pages (from-to)153-166
Number of pages14
JournalActa Neurobiologiae Experimentalis
Volume62
Issue number3
Publication statusPublished - 2002

Keywords

  • Conversion
  • Iatrogenic
  • Infection
  • Prion protein
  • Prions metal-bound
  • Prions plastic-bound
  • Propagation
  • Sterilosation
  • Strains
  • Transmission

ASJC Scopus subject areas

  • Neuroscience(all)

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