Figure 5.

Transmission studies. Transgenic (Tg) HuMM mice challenged with brain homogenate of the MM2‐thalamic patient showed a punctuate and coarse pattern of protease resistant core of the disease‐associated prion protein (PrP^Sc) deposition, mainly confined to the stratum lacunosum of the hippocampus and the cerebral cortex (A,C). The same Tg HuMM mouse line did not show prion protein (PrP) immunoreactivity in the brain after inoculation with brain homogenate of the MM2‐cortical patient (B). Western blot analysis following PTA precipitation did not reveal the presence of PrP^Sc in brain samples from HuMM mice challenged with the MM2‐cortical case (D, lanes 1 and 2) but showed type 2 PrP^Sc in mice inoculated with the MM2‐thalamic case (D, lanes 3 and 4). Lane 5 and lane 6 correspond to sporadic CJD (sCJD) type 1 and type 2 PrP^Sc, respectively, used as controls. Scale bar in C = 100 µm.