Fig. 2From: Familial human prion diseases associated with prion protein mutations Y226X and G131V are transmissible to transgenic mice expressing human prion proteinImmunohistochemistry and neuropathology of tg66 mice injected with Y226X human brain homogenate. Panel a Pons region of a mouse euthanized at 593 dpi. PrPSc was detected by IHC using biotinylated antibody 3F4 as described in the methods (panel a-1). Large and medium-sized PrPSc deposits are seen at higher magnification (a-2). Inset in a-2 shows Thioflavin S staining of one aggregate. Typical prion disease vacuolation (arrow) is shown by H&E staining (a-3), and astrogliosis and microgliosis (arrow) are shown by anti-GFAP staining (a-4) and anti-Iba1 staining (a-5). Panel b Pons region of a mouse euthanized at 601 dpi. PrPSc staining showed smaller coarse deposits (b-1), and perineuronal and linear axonal staining (arrows) could be seen at higher magnification (b-2). Vacuolation, astrogliosis and microgliosis (arrows) was also prominent in this same area (b-3, b-4, b-5). Panel c: Thalamus of same mouse shown in panel b showed slightly finer staining of PrPSc at both low (c-1) and high (c-2) magnification. Prominent vacuolation (c-3), astrogliosis (c-4) and microgliosis (c-5) was also noted (arrows). Scale bars shown in a-1, b-1 and c-1 are 200 μm, scale bars shown in a-2, b-2 and c-2 are 50 μm and apply to each subsequent panel within the same figure letterBack to article page