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Healthy goats naturally devoid of prion protein

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 ce...

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Published in:	Veterinary research (Paris) 2012-12, Vol.43 (1), p.87-87
Main Authors:	Benestad, Sylvie L, Austbø, Lars, Tranulis, Michael A, Espenes, Arild, Olsaker, Ingrid
Format:	Article
Language:	English
Subjects:	Animal genetic engineering Animals Blotting, Western - veterinary Bovine spongiform encephalopathy Codon, Nonsense Creutzfeldt-Jakob disease Enzyme-Linked Immunosorbent Assay - veterinary Genetic aspects Genetically modified animals Goats Goats - genetics Goats - metabolism Medical research Medicine, Experimental Molecular Sequence Data Norway Polymerase Chain Reaction - veterinary Prions Prions - genetics Prions - metabolism Proteases Sequence Analysis, Protein - veterinary Short Report
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creator	Benestad, Sylvie L Austbø, Lars Tranulis, Michael A Espenes, Arild Olsaker, Ingrid
description	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.
doi_str_mv	10.1186/1297-9716-43-87
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subjects	Animal genetic engineering Animals Blotting, Western - veterinary Bovine spongiform encephalopathy Codon, Nonsense Creutzfeldt-Jakob disease Enzyme-Linked Immunosorbent Assay - veterinary Genetic aspects Genetically modified animals Goats Goats - genetics Goats - metabolism Medical research Medicine, Experimental Molecular Sequence Data Norway Polymerase Chain Reaction - veterinary Prions Prions - genetics Prions - metabolism Proteases Sequence Analysis, Protein - veterinary Short Report
title	Healthy goats naturally devoid of prion protein
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