Prion diseases are incurable neurodegenerative conditions affecting both animals and humans. They may be sporadic, infectious, or inherited in origin. Human prion diseases include Creutzfeldt—Jakob desease (CJD), Gerstmann—Straussler—Scheinker disease, kuru, and fatal familial insomnia. The appearance of variant CJD, and the demonstration that is caused by strains indistinguishable from bovine spongiform encephalopathy (BSE) in cattle, has led to the threat of a major epidemic of human prion disease in the UK and other countries where widespread dietary exposure to bovine prions has occurred. This article reviews the history and epidemiology of these diseases, and then focuses on important areas of current research in human prion disorders.
Keywordsbovine spongiform encephalopathy Creutzfeldt—Jakob disease fatal familial insomnia Gerstmann—Straussler—Scheinker syndrome kuru prion scrapie spongiform encephalopathy
Unable to display preview. Download preview PDF.
- Asante EA, Linehan JM, Desbruslais M, Joiner S, Gowland I, Wood A, Welch J, Hill AF, Lloyd SE, Wadsworth JDF, Collinge J (2002). BSE prions propagae as either variant CJD-like or sporadic CJD-like prion strains in transgenic mice expressing human prion protein. EMBO J, 21: 6358–6366.PubMedCrossRefGoogle Scholar
- McGowan JP (1922). Scrapie in sheep. Scott J Agric 5: 365–375.Google Scholar
- Pattison IH (1965). Experiments with scrapie with special reference to the nature of the agent and the pathology of the disease. In: Slow latent and temperate virus infections, NINDB Monograph 2. Gajdusek CJ, Gibbs CJ, Alpers MP (eds). Washington DC: US Government Printing, p. 249–257.Google Scholar
- Prusiner SB et al; Rubin E et al; Stanton BR et al (1990). D 216. Transgenic mice expressing hamster prion protein produce species-specific scrapie infectivity and amyloid plaques. D 217. Production of human hemoglobin S antilles in transgenic mice. D 218. Analysis of murine Nmyc function by in situ hydridization and homologous recombination. J Cellular Biochem UCLA Symposium Suppl 14A: 363.Google Scholar