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Vet Res. 2012 Dec 18;43:87. doi: 10.1186/1297-9716-43-87.

Healthy goats naturally devoid of prion protein.

Author information

1
Norwegian Veterinary Institute, P,O,Box 750, Sentrum, Oslo, 0106, Norway. sylvie.benestad@vetinst.no.

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.

PMID:
23249298
PMCID:
PMC3542104
DOI:
10.1186/1297-9716-43-87
[Indexed for MEDLINE]
Free PMC Article

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