Differentiation of Prions from L-type BSE versus Sporadic Creutzfeldt-Jakob Disease - - Emerging Infectious Disease journal - CDC

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Differentiation of Prions from L-type BSE versus Sporadic Creutzfeldt-Jakob Disease - - Emerging Infectious Disease journal - CDC

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Differentiation of Prions from L-type BSE versus Sporadic Creutzfeldt-Jakob Disease

Article Contents

• The Study
• Conclusions
• Acknowledgments
• References
• Figure 1
• Figure 2
• Table
• Technical Appendix  [284 KB - 3 pages]
• Suggested Citation

Simon Nicot, Anna Bencsik, Eric Morignat, Nadine Mestre-Francés, Armand Perret-Liaudet, and Thierry Baron 

Author affiliations: Author affiliations: Agence Nationale de Sécurité Sanitaire (Anses), Lyon, France (S. Nicot, A. Bencsik, E. Morignat, T. Baron); INSERM U710, Montpellier, France; Université Montpellier 2, Montpellier; École Pratique des Hautes Études, Paris, France (N. Mestre-Francés); Hôpitaux Civils de Lyon, Université Lyon 1, INSERM U1028, and Centre National de la Recherche Scientifique, Lyon (A. Perret-Liaudet)

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Abstract

We compared transmission characteristics for prions from L-type bovine spongiform encephalopathy and MM2-cortical sporadic Creutzfeldt-Jakob disease in the Syrian golden hamster and an ovine prion protein–transgenic mouse line and isolated distinct prion strains. Our findings suggest the absence of a causal relationship between these diseases, but further investigation is warranted.

Among transmissible spongiform encephalopathies (TSEs), the L-type bovine spongiform encephalopathy (L-BSE) in cattle requires particular attention for public health. L-BSE is transmitted more efficiently than is classical BSE among primates (1–3) as well as among transgenic mice that express human prion protein (PrP) (4,5). We recently reported that L-BSE was readily transmissible by experimental oral inoculation in a nonhuman primate species, the grey mouse lemur (Microcebus murinus) (3). These findings raise the possibility that some human Creutzfeldt-Jakob disease (CJD) cases might result from exposure to the L-BSE agent; previous studies highlighted similarities between L-BSE and some human subtypes (type 2) of sporadic CJD (sCJD) (1,6).
To examine the possible relationship between L-BSE and sCJD, we evaluated a strain-typing strategy that relies on comparative transmission characteristics in the Syrian golden hamster and in a transgenic mouse line (TgOvPrP4) expressing ovine PrP (ARQ allele). Both of these species are susceptible to L-BSE prions from cattle (7,8). The transmission of L-BSE, including after a first passage in Microcebus murinus lemurs (3), was compared with that for the MM2-cortical subtype of sCJD (9); this subtype was chosen on the basis of a study that indicated higher levels of molecular similarities of L-BSE with this sCJD subtype than with the MV2 subtype (1).