Transmission of vCJD and BSE to wild-type and transgenic mice: neuropathology and immunocytochemical characterisation

Hudson, L.; Rezaie, P.; Collinge, C. and Lantos, P. L. (2001). Transmission of vCJD and BSE to wild-type and transgenic mice: neuropathology and immunocytochemical characterisation. In: Centenary Meeting of the British Neuropathological Society, 11-12 Jan 2001, London, UK.


Introduction: It has been previously reported that the same `prion strain' causes vCJD and BSE [Hill et al. Nature 1997; 389 : 448]. The scrapie isoform of prion protein (PrP) detected in vCJD has a ratio of glycoforms that closely resembles that of BSE passaged in several species [Collinge et al. Nature 1996; 383 : 685]. FVB mice inoculated with vCJD produced mouse PrPSc with vCJD-like glycoform ratios and fragment sizes indistinguishable from those in FVB mice expressing human PrP [Hill et al. Nature 1997; 389 : 448]. Striking similarities have also been noted in the patterns of PrP deposition between BSE and vCJD-inoculated mice. The aim of this investigation was to assess further the characteristic immunophenotypical patterns of prion deposition associated with transmission of vCJD and BSE to mice. Materials and Methods: Tg152 mice expressing human PrP alone [HuPrP+/+ Prnp0/0] and wild-type FVB mice were inoculated with 30 ml of a 1:10 homogenate of vCJD or BSE-infected CNS material. PBS inoculated animals were used as controls. The brains of these aimals were subsequently screened for deposition of PrP by immunocytochemistry using antibodies 3F4, 12F10 and sp40. Results: The pattern of PrP deposition included diffuse, diffuse-punctate, plaque and pericellular deposits associated with transmission to both wild-type and transgenic mice. Specifically, BSE and vCJD markedly up-regulated pericellular reactivity in transgenic animals; a feature not demonstrable in wild-types. The distribution of PrP was similar in vCJD and BSE, primarily targeting deep grey nuclei, midbrain, medulla and cerebellum Conclusion: These results demonstrate that vCJD and BSE display similar neuropathological and immunophenotypical characteristics of transmission.

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