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

PLoS Pathogens

Thierry Baron¹  Anna Bencsik¹  Anne-Gaëlle Biacabé¹  Nathalie Chenais²  Jean-Luc Vilotte²  Gaëlle Tilly²  Vincent Béringue³  Fabienne Reine³  Annick Le Dur³  Thanh Lan Laï³  Hubert Laude³ 

[1] Agence Française de Sécurité Sanitaire des Aliments, Unité Agents Transmissibles Non Conventionnels, Lyon, France;Institut National de la Recherche Agronomique, Génétique Biochimique, et Cytogénétique, Jouy-en-Josas, France;Institut National de la Recherche Agronomique, Virologie Immunologie Moléculaires, Jouy-en-Josas, France

关键词: Cattle;    Animal prion diseases;    Bovine spongiform encephalopathy;    Sheep;    Spleen;    Mouse models;    Hippocampus;    Bovines;   

DOI  :  10.1371/journal.ppat.0020112

学科分类：生物科学（综合）

来源: Public Library of Science

【 摘 要 】

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 (PrPres) isolated from brains of affected animals or humans. However, alternate PrPres 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 PrPres 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.

【 授权许可】

CC BY