Healthy goats naturally devoid of prion protein |
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Authors: | Sylvie L Benestad Lars Austb? Michael A Tranulis Arild Espenes Ingrid Olsaker |
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Institution: | 1.Norwegian Veterinary Institute, P.O.Box 750, Sentrum, Oslo, 0106, Norway;2.The Norwegian School of Veterinary Science, P.O.Box 8146 Dep, Oslo, 0033, Norway |
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Abstract: | 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. |
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