Characterization of twelve French Nor98 isolates in the TgOvPrP4 mouse model

摘要

Atypical scrapie or Nor98 has been identified as a transmissible spongiform en-cephalopathy, clearly discernible from classical scrapie and BSE, notably regarding the biochemical features of the protease-resistant prion protein (PrPres) and the genetic factors involved in susceptibility to the disease. Recently our group reported successful transmission of such isolates to the transgenic mouse model (TgOvPrP4) overexpressing the ovine PrPARQ protein. Here, we report the histopathological study of the brains of TgOvPrP4 transgenic mice infected with twelve French cases of atypical scrapie. The uniform histopathological features found in TgOvPrP4 mice infected with the twelve different atypical scrapie sources confirm the uniform biochemical features reported previously and contribute to the characterisation of the Nor98 strain.