Healthy goats naturally devoid of prion protein

被引：0
作者：
Sylvie L Benestad
Lars Austbø
Michael A Tranulis
Arild Espenes
Ingrid Olsaker
机构：
[1] Norwegian Veterinary Institute,
[2] The Norwegian School of Veterinary Science,undefined
来源：
Veterinary Research | / 43卷
关键词：
Prion Protein; Prion Disease; Bovine Spongiform Encephalopathy; Scrapie; Classical Scrapie;
D O I：
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学科分类号：
摘要：
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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