Fig. 2: The number of PML-NBs is reduced in lumbar spinal cord α-MNs of C9orf72 ALS-FTD and FUS ALS-FTD (R521C).
From: Loss of PML nuclear bodies in familial amyotrophic lateral sclerosis-frontotemporal dementia
A DAB immunolabelling using PML antibody (paraffin sections) on the lumbar spinal cord. α-MNs of a representative control section (upper panel) show strong nuclear immunoreactivity of PML, whereas α-MNs of C9orf72 ALS-FTD (middle panel) and of FUS-ALS-FTD (lower panel) show an overall drastically reduced nuclear immunoreactivity. n = 3 control, n = 4 C9orf72-ALS-FTD, n = 3 FUS-ALS-FTD. Scale bar is 20 μm. B, C Co-immunolabeling using phosphorylated TDP-43 (pTDP-43) and PML antibodies in the lumbar spinal cord α-MNs of a representative control section (upper panel) and C9orf72 ALS-FTD (lower panel). The white arrows point to nuclear PML immunoreactivity (B). PML-NBs were automatically segmented and quantified. B The average number of PML-NBs/nucleus is shown. Number of lumbar spinal cord a-MNs analyzed: control (24); C9orf72 ALS-FTD (45); staining p-TDP43 and PML. C Note the strongly reduced immunolabelling of PML-NBs in the α-MNs harboring pTDP-43 aggregates compared to the adjacent α-MNs without any pTDP-43 aggregates. Serial paraffin sections; scale bar is 40 μm (zoom:20 μm). n = 3 control, n = 4 C9orf72-ALS-FTD. D Co-immunolabeling using FUS and PML antibodies in the lumbar spinal cord α-MNs. The white arrow shows lack of nuclear PML immunoreactivity in the α-MNs harboring FUS aggregates. The average number of PML-NBs/nucleus is shown. Serial paraffin sections; scale bar is 30 μm (zoom:15 μm). Number of lumbar spinal cord a-MNs analyzed: control (12); FUS-ALS (R521C) (15); staining FUS and PML.
