Additional file 3: Figure S3. of Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

Immunohistochemical detection of pIRE1α and pPERK in AD and CJD hippocampus. Immunohistochemical detection for pIRE1α and pPERK was performed on an AD (case #57) and CJD (case #42) brain tissue with identical post-mortem handling and delay. Pictures from the subiculum are shown. Immunohistochemical detection is visualized by DAB (brown staining) and nuclei are counterstained with haematoxylin (blue staining). Bar 50 μm. (TIF 21347 kb

Overview of used antibodies.

<p>Overview of used antibodies.</p

Additional file 2: Figure S2. of Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

Immunohistochemical detection of PrP, pIRE1α, pPERK, and CK1δ in brain tissue of various human prion disease subtypes. Representative pictures of the immunohistochemical detection of PrP (3F4 antibody), pIRE1α, pPERK and CK1δ in frontal cortex sections of human prion disease patients with different disease subtypes, namely GSS (case #10), VPSPr (case #56), sCJD (case #27), panencephalopatic CJD (case #55), PrP-CAA (case #15), FFI (case #18), vCJD (case #21) and iCJD (#24), showing the absence of these UPR activation and GVD markers in the presence of PrP deposition. Immunohistochemical detection is visualized by DAB (brown staining) and nuclei are counterstained with haematoxylin (blue staining). Bar 200 μm. (PDF 2131 kb

Additional file 1: Figure S1. of Activation of the unfolded protein response and granulovacuolar degeneration are not common features of human prion pathology

Semi-quantitative analysis of the Aβ (IC16) and phosphorylated tau (AT8) burden. Representative examples from our cohort showing the amount of Aβ deposits, small phosphorylated tau positive extracellular inclusions and neuritic changes related to prion disease and phosphorylated tau positive tangle-like structures, most often resulting from AD pathology, corresponding to the semi-quantitative scores +, ++ and +++. Aβ images were taken with the 5× objective, tau (positive inclusions/neuritic changes) images with the 10× objective, tau (tangle-like changes) images with the 20× objective. In Table 3 scores for each case are listed. Scores with a/, e.g. +/++, have a burden in-between the classes represented here. Brown staining with DAB, blue staining of the nucleus with haematoxylin. (PDF 1437 kb

Patient characteristics.

<p>Patient characteristics.</p

Results on infiltrating immune cells and Granzyme inhibitor expression of the individual patients.

<p>Results on infiltrating immune cells and Granzyme inhibitor expression of the individual patients.</p

Immune (evasion) markers in cases of CNS-PNET.

<p>Immunohistochemical staining of immune markers in two CNS-PNET cases. Note that SerpinB9 expression coincides with high numbers of activated GrB ⁺ CTLs in case 1 versus low numbers of activated GrB ⁺ CTLs in case 2. Size bar equals 50μm.</p

Additional file 1: Figure S1. of Specific pattern of maturation and differentiation in the formation of cortical tubers in tuberous sclerosis complex (TSC): evidence from layer-specific marker expression

A. Loss of neurons throughout all cortical layers in TSC cortical tubers and FCD type IIb. B. A larger amount of dysmorphic neurons can be detected in FCD type IIb compared to TSC cortical tubers. C. Significant increase in heterotopic neurons in the deep white matter in patients who underwent surgery for FCD Type IIb or cortical tuber resection. D. Quantitative analysis of Satb2+ neurons revealed significant overall cell loss in all epilepsy surgery specimens. E. Relative loss of Satb2+ neurons in TSC tissue. F. Quantitative increase in Tbr1+ neurons in perilesional cortex (PLx). G. Quantitative increase of Tbr1+ cells in the upper cortical layers. (TIF 26642 kb