Healthy goats naturally devoid of prion protein

作者：Benestad Sylvie L^*; Austbo Lars; Tranulis Michael A; Espenes Arild; Olsaker Ingrid

来源：Veterinary Research, 2012, 43: 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.

• 出版日期2012-12-18