Protease-Sensitive Synthetic Prions (original) (raw)
Figure 4
Protease-sensitive synthetic prions are serially transmissible to Tg4053 mice.
Tg4053 mice intracerebrally inoculated with MoSP2-1T (red), MoSP19A (blue) or MoSP19B (purple) developed signs of prion disease between 600–750 d (A). MoSP19A and MoSP19B are two Tg9949 brain isolates inoculated with Amyloid Prep 19 (origin in Table S3). Tg4053 mice inoculated with protease-sensitive synthetic prions were significantly more likely to develop neurological dysfunction than mice inoculated with uninfected Tg9949 brain homogenate (black, p<0.001). PrP in the brains of these mice was sensitive to PK digestion (B) and active in the ASA (C). MoSP1 was used as a control. The brains of ill, MoSP2-inoculated Tg4053 mice showed neuropathology consistent with prion disease (D), including vacuolation (top panel), astrocytic gliosis (middle panel), and punctate PrP deposits (bottom panel). Scale bars represent 100 µm.