Alba Marín-Moreno
1, Alvina Huor
1, Juan Carlos Espinosa, Jean Yves Douet, Patricia Aguilar-Calvo
2, Naima Aron, Juan Píquer, Sévérine Lugan, Patricia Lorenzo, Cecile Tillier, Hervé Cassard, Olivier Andreoletti, and Juan María Torres
Figure 2. Atypical BSE transmission into sheep PrP transgenic mice in a study of atypical BSE transmission using isolates from different countries in Europe and transgenic mouse models overexpressing human normal brain prion protein. A) Brain PrPres profile of L-BSE prions (lane 2) changed once passaged into TgVRQ (lane 3) and TgARQ (lane 5). L-BSE propagation into TgVRQ and TgARQ produced a PrPres profile with a molecular weight slightly higher than C-BSE (lane 1). L-BSE/TgVRQ transmission into TgVal129 mice rendered a PrPres similar to type 2 sCJD profile when transmitted in the same animal model (lane 4). All inoculated animals were analyzed and individual variations in the PrPres profile among them were not found. Lane 1, C-BSE2; lane 2, BSE L2; lane 3, BSE L2/TgVRQ; lane 4, BSE L2/TgVRQ/TgVal129; lane 5, BSE L2/TgARQ. B) Brain PrPres profile of H-BSE prions (lane 2) changed once passaged into TgVRQ (lane 3) and produced a 21 kDa PrPres profile very different from that of C-BSE (lane 1). H-BSE/TgVRQ transmission into TgMet129 (lane 4) and TgVal129 (lane 5) mice rendered a PrPres similar to type 1 sCJD profile when transmitted in the same animal models. All inoculated animals were analyzed and individual variations in the PrPres profile among them were not found. Lane 1, C-BSE2; lane 2, BSE H3; lane 3, BSE H3/TgVRQ; lane 4, BSE H3/TgVRQ/TgMet129; lane 5, BSE H3/TgVRQ/TgVal129. BSE, bovine spongiform encephalopathy; C-BSE, classical bovine spongiform encephatlopathy; PrP, prion protein; PrPres, proteinase K–resistant prion protein; sCJD, sporadic Creutzfeldt-Jakob disease.