Healthy goats naturally devoid of prion protein

<p>Abstract</p> <p>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-...

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Bibliographic Details
Main Authors: Benestad Sylvie L, Austbø Lars, Tranulis Michael A, Espenes Arild, Olsaker Ingrid
Format: Article
Language:English
Published: BMC 2012-12-01
Series:Veterinary Research
Online Access:http://www.veterinaryresearch.org/content/43/1/87
Description
Summary:<p>Abstract</p> <p>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.</p>
ISSN:0928-4249
1297-9716