Ovine prion protein variant A(136)R(154)L(168)Q(171) increases resistance to experimental challenge with bovine spongiform encephalopathy agent

Wilfred Goldmann, Fiona Houston, Paula Stewart, Matteo Perucchini, James Foster, Nora Hunter

Research output: Contribution to journalArticlepeer-review

Abstract / Description of output

Susceptibility and incubation periods of transmissible spongiform encephalopathies, such as scrapie in sheep, are modulated by the PrP gene. The standard model of association between ovine PrP genetics and classical scrapie susceptibility is based on PrP genotypes with respect to codons 136, 154 and 171, e.g. alanine-arginine-glutamine (ARQ). It is demonstrated here that a proline to leucine substitution in codon 168 of the ovine PrP protein gene is associated with increased resistance to experimental bovine spongiform encephalopathy (BSE) inoculation. The ARL(168)Q PrP allele was found in heterozygous ARP(168)Q/ARL(168)Q sheep that have so far survived intravenous BSE challenge three times longer than BSE-challenged homozygous ARP(168)Q/ARP(168)Q sheep, which develop disease in around 700 days. In contrast, the L141F polymorphism does not appear to be associated with susceptibility to intravenous BSE challenge.
Original languageEnglish
Pages (from-to)3741-5
Number of pages5
JournalJournal of General Virology
Volume87
Issue numberPt 12
DOIs
Publication statusPublished - Dec 2006

Keywords / Materials (for Non-textual outputs)

  • Alleles
  • Amino Acid Substitution
  • Animals
  • Cattle
  • Disease Models, Animal
  • Disease Susceptibility
  • Encephalopathy, Bovine Spongiform
  • Genotype
  • Heterozygote
  • Immunity, Innate
  • Polymorphism, Genetic
  • Prion Diseases
  • Prions
  • Sheep
  • Survival

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