Healthy goats naturally devoid of prion protein

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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. © 2012 Benestad et al.; licensee BioMed Central Ltd.




Benestad, S. L., Austbø, L., Tranulis, M. A., Espenes, A., & Olsaker, I. (2012). Healthy goats naturally devoid of prion protein. Veterinary Research, 43(1).

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