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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Bibliographic Details
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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Summary: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.
ISSN:1297-9716
0928-4249
1297-9716
DOI:10.1186/1297-9716-43-87