The role of host PrP in Transmissible Spongiform Encephalopathies

Enrico Cancellotti, Rona M Barron, Matthew T Bishop, Patricia Hart, Frances Wiseman, Jean C Manson

Research output: Contribution to journalArticlepeer-review


PrP has a central role in the Transmissible Spongiform Encephalopathies (TSEs), and mutations and polymorphisms in host PrP can profoundly alter the host's susceptibility to a TSE agent. However, precisely how host PrP influences the outcome of disease has not been established. To investigate this we have produced by gene targeting a series of inbred lines of transgenic mice expressing different PrP genes. This allows us to study directly the influence of the host PrP gene in TSEs. We have examined the role of glycosylation, point mutations, polymorphisms and PrP from different species on host susceptibility and the disease process both within the murine species and across species barriers.
Original languageEnglish
Pages (from-to)673-80
Number of pages8
JournalBBA - Proteins and Proteomics
Issue number6
Publication statusPublished - 2007


  • Animals
  • Genetic Predisposition to Disease
  • Glycosylation
  • Mice
  • Point Mutation
  • Polymorphism, Genetic
  • Prion Diseases
  • Prions


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