Fig. 1: The number of PML-NBs is reduced in the frontal cortex and hippocampus of C9orf72 ALS-FTD patients.
From: Loss of PML nuclear bodies in familial amyotrophic lateral sclerosis-frontotemporal dementia
A DAB immunolabelling using PML antibody (paraffin sections) on the frontal cortex and dentate gyrus (hippocampus) of a representative control section (upper panel), showing strong nuclear immunoreactivity of PML, of C9orf72 ALS-FTD (middle panel) and of FUS-ALS-FTD (lower panel), showing an overall reduced nuclear immunoreactivity. n = 3 control, n = 4 C9orf72-ALS-FTD, n = 3 FUS-ALS-FTD. Scale bar is 50 μm. Co-immunolabeling using poly-GA and PML antibodies showing a significant decrease of PML-NB number in hippocampal dentate gyrus (B) and cortical (C) neurons harboring polyGA aggregates in C9orf72 ALS-FTD brain, compared to the controls (upper panel). n = 3 control, n = 4 C9orf72-ALS-FTD, n = 3 FUS-ALS-FTD. Scale bar is 20 μm. PML and nuclei were segmented using ScanR (Olympus) and the number and size of PML-NBs per nucleus was automatically quantified. Quantification of PML-NB average number and size (px^2) is shown. Number of cells analyzed: dentate gyrus control (1263); dentate gyrus C9orf72 ALS-FTD (1523) (B); frontal cortex control (122); frontal cortex C9orf72 ALS-FTD (682) (C).
