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Figure 2 | Acta Neuropathologica Communications

Figure 2

From: Rapidly progressive dementia with thalamic degeneration and peculiar cortical prion protein immunoreactivity, but absence of proteinase K resistant PrP: a new disease entity?

Figure 2

Immunostaining for PrP. Immunostaining for PrP revealed tiny granular deposits depicting neuronal processes in a linear or worm-like appearance predominantly in the 2nd and 3rd layers in neocortical regions. (a-c; representative images of case 2; right upper inset in c shows similar immunoreactivity in the cerebellum in case 3). Occasionally, small cell bodies were depicted as well (d). These were detectable also by antibodies 6H4 (e), and KG9 (f), only focally by 3F4 (g), and not by the anti-N-terminal BG4 antibody (h all representative images of case 4). The PrP immunoreactivity (i) was abolished after PK treatment (j; temporal cortex of case 1), while it was slightly more detectable when using only elongated citrate buffer pretreatment and omitting formic acid pretreatment (k: with, and l: without formic acid, represented by the occipital cortex of case 5). PET-blot examination performed using Thermolysin digested PrPC completely from the frontal cortex brain tissue of a non-CJD control case (m), while in the present cases the worm-like structures (enlarged in right upper inset) remained visible (n), and in the cortex of a subject with CJD abundant PrPSc deposition was seen (o). Bar in a represents 150 μm for a, 50 μm for b, e-l, 30 μm for c, 10 μm for d, and 400 μm for m-o.

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