Atypical prion diseases in humans and animals.

  title={Atypical prion diseases in humans and animals.},
  author={Michael A Tranulis and Sylvie L. Benestad and Thierry Baron and Hans Kretzschmar},
  journal={Topics in current chemistry},
Although prion diseases, such as Creutzfeldt-Jakob disease (CJD) in humans and scrapie in sheep, have long been recognized, our understanding of their epidemiology and pathogenesis is still in its early stages. Progress is hampered by the lengthy incubation periods and the lack of effective ways of monitoring and characterizing these agents. Protease-resistant conformers of the prion protein (PrP), known as the "scrapie form" (PrP(Sc)), are used as disease markers, and for taxonomic purposes… CONTINUE READING


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