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.