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Characterization of atypical scrapie cases from Great Britain in transgenic ovine PrP mice

  • Peter C. Griffiths
  • , John Spiropoulos
  • , Richard Lockey
  • , Anna C. Tout
  • , Dhanushka Jayasena
  • , Jane M. Plater
  • , Alun Chave
  • , Robert B. Green
  • , Sarah Simonini
  • , Leigh Thorne
  • , Ian Dexter
  • , Anne Balkema-Buschmann
  • , Martin H. Groschup
  • , Vincent Béringue
  • , Annick Le Dur
  • , Hubert Laude
  • , James Hope

Research output: Contribution to journal › Article › peer-review

36 Citations (Scopus)

Abstract

Twenty-four atypical scrapie cases from sheep with different prion protein genotypes from Great Britain were transmitted to transgenic tg338 and/or TgshpXI mice expressing sheep PrP alleles, but failed to transmit to wild-type mice. Mean incubation periods were 200-300 days in tg338 mice and 300-500 days in TgshpXI mice. Survival times in C57BL/6 and VM/Dk mice were >700 days. Western blot analysis of mouse brain samples revealed similar multi-band, protease-resistant prion protein (PrPres) profiles, including an unglycosylated band at ∼8-11 kDa, which was shown by antibody mapping to correspond to the ∼93-148 aa portion of the PrP molecule. In transgenic mice, the incubation periods, Western blot PrPres profiles, brain lesion profiles and abnormal PrP (PrPSc) distribution patterns produced by the Great Britain atypical scrapie isolates were similar and compatible with the biological characteristics of other European atypical scrapie or Nor98 cases.

Original languageEnglish
Pages (from-to)2132-2138
Number of pages7
JournalJournal of General Virology
Volume91
Issue number8
DOIs
Publication statusPublished - Aug 2010

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