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Fig. 3 | Acta Neuropathologica Communications

Fig. 3

From: Impaired β-glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations

Fig. 3

Lower GCase Immunolabeling in FTD-GRN. We performed GCase immunostaining with the rabbit polyclonal antibody used in the preceding biochemical experiments and a second mouse monoclonal antibody. a, using homozygous GBA L444P fibroblasts, we confirmed that the rabbit polyclonal antibody detected an immature form of GCase (also see Additional file 2: Figure S2), but the mouse monoclonal antibody was selective for mature, fully glycosylated GCase. We analyzed GCase immunolabeling in each group in layer III, which contained many strongly-labeled pyramidal neurons in controls. b, c, FTD-GRN patients did not exhibit significantly less GCase immunolabeling with the rabbit polyclonal antibody (t test, p = 0.2042). d, e, In contrast, FTD-GRN patients had significantly less GCase immunolabeling with the mouse monoclonal antibody selective for mature GCase (t test, p = 0.0285). Low power images of GCase immunoreactivity throughout the cortex are shown in b and d, with 100 μm scale bars, with representative 20x images of GCase immunostaining below with a 20 μm scale bars. The numbers for each image reference the patients described in Table 1

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