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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: Bona fide atypical scrapie faithfully reproduced for the first time in a rodent model

Fig. 2

Brain lesions and PrPres deposit distribution for TgShI112 mice. a Line 456 (n = 14) and b Line L460 (n = 7). Brain lesion profiles and PrPres deposition profiles represent the mean semi-quantitative scoring (0–4, vertical axis) of the spongiform lesions (continuous line, black) and the immunohistochemical labelling of PrPres deposits (dashed line, grey) against 14 brain regions (Pfc: piriform cortex, H: hippocampus, Oc: occipital cortex, Tc: temporal cortex, Pc: parietal cortex, Fc: frontal cortex, cc: corpus callosum; S: striatum, T: thalamus, HT: hypothalamus, M: mesencephalon, Mob: medulla oblongata, Cm: cerebellar nuclei, Cv: cerebellar vermis, Cc: cerebellar cortex). Lesions in line L460 are milder than those of L456 but follow a similar distribution. Bars: standard error of the mean. c Histopathological assessment of spongiform lesions and PrPres deposits in TgShI112 mice (line 456).Spongiform change can be observed in the top row (H&E staining). PrPres deposits are of punctiform and granular nature. In the bottom row a healthy L456 mouse brain is shown. Notice the intense PrPC backroad in the cerebellar molecular layer and the synaptic glomerules (but lacking the punctiform PrPres staining pattern). Mouse monoclonal antibody 2G11 (1:100). d Neuroinflammatory response. The animals that develop a disease also show a conspicuous neuroinflammatory response with activation of both astroglial (Glial fibrillary acidic protein –GFAP- immunohistochemistry, 1:1500) and microglial (Lycopersicum esculentum agglutinin –LEA- affinity histochemistry, 1:50) cell populations in comparison to age matched unaffected siblings. e Western blotting of a classical scrapie isolate (SSBP/1) and a healthy sheep brain homogenate (Ov NBH) digested for the detection of classical PrPres, compared to an isolate of atypical (Nor98) ovine scrapie and the prions extracted from spontaneously diseased TgShI112 mice digested according to the protocol described in [78], which allows detection of atypical PrPSc pattern, and requires 20 times more brain homogenate. The same undigested samples from spontaneously diseased TgShI112 mice and atypical scrapie were also included. Anti PrP antibody 12B2 (1:5000). Spontaneous TgShI112 displays an atypical ladder-like pattern resembling the Nor98 atypical scrapie isolate. MW: Molecular weight

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