%0 Journal Article
%T Healthy goats naturally devoid of prion protein
%A Benestad Sylvie L
%A Austb£¿ Lars
%A Tranulis Michael A
%A Espenes Arild
%J Veterinary Research
%D 2012
%I BioMed Central
%R 10.1186/1297-9716-43-87
%X 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.
%U http://www.veterinaryresearch.org/content/43/1/87