Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate

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Abstract

Background: Human variant Creutzfeldt-Jakob Disease (vCJD) results from foodborne transmission of prions from slaughtered cattle with classical Bovine Spongiform, Encephalopathy (cBSE).forms of B5E, which remain mostly asymptornatic in aging cattle, were recently identified at slaughterhouses throughout Europe and North Americo, raising a question about human susceptibility to these new prion strains. Methodology/Principal Findings: Brain homogenates from cattle with classical BSE and atypical (BASE) infections were inoculated intracerebrally into cynomolgus monkeys (Macacca fascicularis), a non-human primate model previously demonstrated to be susceptible to the original strain of cBSE. The resulting diseases were compared in terms of clinical signs, histology and biochemistry of the abnormal prion protein (PrPres), The single monkey infected with BASE had a shorter survival, and a different clinical evolution, histopathology, and prion protein (PrPres) pattern than was observed for either classical BSE or vCID-inoculated animals. Also, the biochemical signature of PrPres in the BASE-inoculated animal was found to: have a higher proteinase K sensitivity of the octa-repeat region. We found the same biochemical signature in three of four human patients With sporadic CJD and an MM type 2 PrP genotype who lived in the same country as the infected bovine. Conclusion/Significance: Our results point to a possibly higher degree of pathogenicity of BASE than classical BSE in primates and also raise a question about a possible link to one uncommon subset of cases of apparently sporadic CJD. Thus, despite the waning epidemic of classical BSE, the occurrence of atypical strains should temper the urge to relax measures currently in place to protect public health from accidental contamination by BSE-contaminated products. © 2008 Comoy et al.

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Comoy, E. E., Casalone, C., Lescoutra-Etchegaray, N., Zanusso, G., Freire, S., Marcé, D., … Deslys, J. P. (2008). Atypical BSE (BASE) transmitted from asymptomatic aging cattle to a primate. PLoS ONE, 3(8). https://doi.org/10.1371/journal.pone.0003017

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