(A) Comparison of neuropathological features including PrPSc deposition, spongiotic changes (presented in H&E stainings), astrocyte (GFAP) and microglia (Iba-1) activation in striatum (as a site in close proximity to prion inoculation) and cerebellum (resembling a brain area distant to prion inoculation) in A10 cKO and littermate control mice at a terminal stage of prion disease (i.e., ∼103 days post inoculation [dpi] for A10 cKO and ∼146 dpi for controls). While similar pathological alterations were observed in the striatum of both genotypes, prion-associated lesions in the cerebellum were almost absent in A10 cKO mice. (B) At a matched time point (95 dpi for A10 cKO and littermate controls; 65 dpi [i.e., terminal disease] for tga20), prion-related pathology was found in the striatum of all genotypes analyzed. As described earlier for the cortex and hippocampus (Figure 6), differences in PrPSc amounts could also be observed in the striatum. In the cerebellum of both time-matched A10 cKO mice and littermate controls, prion-associated lesions were largely absent whereas tga20 mice showed all relevant neuropathological features already at 65 dpi. Representative pictures for at least three animals per genotype and time point are shown. Scale bars represent 200 µm (overviews) or 100 µm (insets).