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.
Béringue
Vincent
V
Institut National de la Recherche Agronomique, Virologie Immunologie Moléculaires, Jouy-en-Josas, France.
Bencsik
Anna
A
Le Dur
Annick
A
Reine
Fabienne
F
Laï
Thanh Lan
TL
Chenais
Nathalie
N
Tilly
Gaëlle
G
Biacabé
Anne-Gaëlle
AG
Baron
Thierry
T
Vilotte
Jean-Luc
JL
Laude
Hubert
H
D000818:Animals; D001921:Brain / Q000378:metabolism* / Q000473:pathology; D002417:Cattle; D004198:Disease Susceptibility; D016643:Encephalopathy, Bovine Spongiform / Q000378:metabolism* / Q000473:pathology / Q000635:transmission; D008136:Longevity; D051379:Mice; D008822:Mice, Transgenic; D018620:PrPSc Proteins / Q000302:isolation & purification* / Q000378:metabolism*; D012756:Sheep; D013045:Species Specificity
D018620:PrPSc Proteins