A Systematic Review of Prion Therapeutics in Experimental Models

Brain. 2006 Sep;129(Pt 9):2241-65. doi: 10.1093/brain/awl150. Epub 2006 Jul 1.


Prion diseases are transmissible, invariably fatal, neurodegenerative diseases which include Creutzfeldt-Jakob disease (CJD) in humans and bovine spongiform encephalopathy and scrapie in animals. A large number of putative treatments have been studied in experimental models over the past 30 years, with at best modest disease-modifying effects. The arrival of variant CJD in the UK in the 1990s has intensified the search for effective therapeutic agents, using an increasing number of animal, cellular and in vitro models with some recent promising proof of principle studies. Here, for the first time, we present a comprehensive systematic, rather than selective, review of published data on experimental approaches to prion therapeutics to provide a scientific resource for informing future therapeutics research, both in laboratory models and in clinical studies.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Animals
  • Anti-Bacterial Agents / therapeutic use
  • Anticoagulants / therapeutic use
  • Antimalarials / therapeutic use
  • Antiviral Agents / therapeutic use
  • Cattle
  • Congo Red / therapeutic use
  • Creutzfeldt-Jakob Syndrome / drug therapy
  • Creutzfeldt-Jakob Syndrome / immunology
  • Disease Models, Animal
  • Encephalopathy, Bovine Spongiform / drug therapy
  • Encephalopathy, Bovine Spongiform / immunology
  • Glycosaminoglycans / therapeutic use
  • Humans
  • Immunotherapy / methods
  • Polyamines / therapeutic use
  • PrPC Proteins / drug effects
  • Prion Diseases / drug therapy*
  • Prion Diseases / immunology
  • Tetracyclines / therapeutic use


  • Anti-Bacterial Agents
  • Anticoagulants
  • Antimalarials
  • Antiviral Agents
  • Glycosaminoglycans
  • Polyamines
  • PrPC Proteins
  • Tetracyclines
  • Congo Red