Prions: disease propagation and disease therapy by conformational transmission

Trends Mol Med. 2001 Mar;7(3):109-14. doi: 10.1016/s1471-4914(01)01931-1.

Abstract

Transmissible spongiform encephalopathies - also known as prion-related diseases - are a group of fatal neurodegenerative disorders associated with the misfolding of prion protein. Several unprecedented scientific findings, which have directly confronted popular dogmas in biology, have put prion research in the spotlight. The experimental evidence supports an entirely novel disease mechanism, involving disease transmission by replication of protein conformation. Here, we describe exciting scientific findings that make the prion field attractively heretical, and we propose the transmission of protein conformation as a novel approach to producing drugs to combat a variety of diseases.

Publication types

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

MeSH terms

  • Animals
  • Humans
  • Prion Diseases / genetics
  • Prion Diseases / therapy*
  • Prion Diseases / transmission
  • Prions / chemistry*
  • Protein Conformation*

Substances

  • Prions