Healthy goats naturally devoid of prion protein

Vet Res. 2012 Dec 18;43(1):87. doi: 10.1186/1297-9716-43-87.


Prion diseases such as scrapie in small ruminants, bovine spongiform encephalopathy (BSE) in cattle and Creutzfeldt-Jakob disease (CJD) in man, are fatal neurodegenerative disorders. These diseases result from the accumulation of misfolded conformers of the host-encoded prion protein (PrP) in the central nervous system. To date naturally-occurring PrP free animals have not been reported. Here we describe healthy non-transgenic animals, Norwegian Dairy Goats, lacking prion protein due to a nonsense mutation early in the gene. These animals are predicted to be resistant to prion disease and will be valuable for research and for production of prion-free products.

MeSH terms

  • Animals
  • Blotting, Western / veterinary
  • Codon, Nonsense*
  • Enzyme-Linked Immunosorbent Assay / veterinary
  • Goats / genetics
  • Goats / metabolism
  • Molecular Sequence Data
  • Norway
  • Polymerase Chain Reaction / veterinary
  • Prions / genetics*
  • Prions / metabolism
  • Sequence Analysis, Protein / veterinary


  • Codon, Nonsense
  • Prions

Associated data

  • GENBANK/KC145281