Fig 2.

PrP immunohistochemistry A: Intense and widespread PrP immunostain of the cerebral cortex and (B) distinctive PrP immunostaining pattern in the hippocampal gyrus with staining of the molecular layers (arrows) but not of the pyramidal cell layer or of the end-plate (*). C and D: The cortical staining consists of coarse granules forming loose clusters with larger granules or a tighter aggregate of granules at the center; D inset: heavily stained globular structures are also present; (A – D: PSPr). E and F: Immunostaining patterns of the cerebral cortex in sCJDVV2 (E) and sCJDVV1 (F) showing laminar staining and occasional perineuronal staining in sCJDVV2 and very weak and fine widespread staining in sCJDVV1. G: No immunostaining is detectable in the cerebral cortex of a non-prion disease control. H – J: Cerebellar immunostaining patterns in PSPr (H), sCJDVV2 (I) and sCJDVV1 (J). There is intense and exclusive staining of large granules in the molecular layers in PSPr (H), presumably corresponding to the eosinophilic micro-structures surrounded by a pale halo shown in Fig 1D; staining of irregular deposit limited to the granule cell layer in sCJDVV2 (I); no detectable staining in sCJDVV1 (the staining of the granule cell nuclei is non-specific) (J). (A – I: mAb 3F4).