Different levels of PrPScΔOR accumulated in the brains of Tg(PrPΔOR)/Prnp0/0 mice infected with RML, 22L, and BSE prions at terminal stages. Western blotting with 6D11 anti-PrP Ab of the brains of terminally ill WT (n = 3) and Tg(PrPΔOR)/Prnp0/0 mice (n = 3) infected with RML (A), 22L (B), and BSE (C) prions after treatment with (+) or without (−) PK. The right panels show levels of PrPScΔOR in Tg(PrPΔOR)/Prnp0/0 mice compared to PrPSc in WT mice. AU, arbitrary units; ns, not significant; *, P < 0.05; **, P < 0.01.