title: Healthy goats naturally devoid of prion protein.
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.
authors.affiliation: Norwegian Veterinary Institute, P,O,Box 750, Sentrum, Oslo, 0106, Norway. sylvie.benestad@vetinst.no.
authors.forename: Sylvie L
authors.initials: SL
authors.lastname: Benestad
authors.forename: Lars
authors.initials: L
authors.lastname: Austbø
authors.forename: Michael A
authors.initials: MA
authors.lastname: Tranulis
authors.forename: Arild
authors.initials: A
authors.lastname: Espenes
authors.forename: Ingrid
authors.initials: I
authors.lastname: Olsaker
mesh_terms: D000818:Animals; D015153:Blotting, Western / Q000662:veterinary; D018389:Codon, Nonsense; D004797:Enzyme-Linked Immunosorbent Assay / Q000662:veterinary; D006041:Goats / Q000235:genetics / Q000378:metabolism; D008969:Molecular Sequence Data; D009664:Norway; D016133:Polymerase Chain Reaction / Q000662:veterinary; D011328:Prions / Q000235:genetics* / Q000378:metabolism; D020539:Sequence Analysis, Protein / Q000662:veterinary
chemical_list: D018389:Codon, Nonsense; D011328:Prions