Isolation from Cattle of a Prion Strain Distinct from That Causing Bovine Spongiform Encephalopathy


To date, bovine spongiform encephalopathy (BSE) and its human counterpart, variant Creutzfeldt-Jakob disease, have been associated with a single prion strain. This strain is characterised by a unique and remarkably stable biochemical profile of abnormal protease-resistant prion protein (PrP(res)) isolated from brains of affected animals or humans. However, alternate PrP(res) signatures in cattle have recently been discovered through large-scale screening. To test whether these also represent separate prion strains, we inoculated French cattle isolates characterised by a PrP(res) of higher apparent molecular mass--called H-type--into transgenic mice expressing bovine or ovine PrP. All mice developed neurological symptoms and succumbed to these isolates, showing that these represent a novel strain of infectious prions. Importantly, this agent exhibited strain-specific features clearly distinct from that of BSE agent inoculated to the same mice, which were retained on further passage. Moreover, it also differed from all sheep scrapie isolates passaged so far in ovine PrP-expressing mice. Our findings therefore raise the possibility that either various prion strains may exist in cattle, or that the BSE agent has undergone divergent evolution in some animals.

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@article{Bringue2006IsolationFC, title={Isolation from Cattle of a Prion Strain Distinct from That Causing Bovine Spongiform Encephalopathy}, author={Vincent B{\'e}ringue and Anna A. Bencsik and Annick Le Dur and Fabienne Reine and Thanh Lan La{\"{i} and Nathalie Chenais and Ga{\"{e}lle Tilly and Anne-Ga{\"{e}lle Biacab{\'e} and Thierry Baron and Jean-luc Vilotte and Hubert Laude}, journal={PLoS Pathogens}, year={2006}, volume={2}, pages={519 - 550} }