Fig. 5

Disruption of SUN proteins in sALS and C9-ALS brain postmortem specimens. a-f. Sections of the brain motor cortex from control (a, d), sALS (b, e) and C9-ALS (c, f) patients were stained with antibodies specific for SUN1 (a-c) and SUN2 (d-f). Hematoxylin and eosin counterstains were used to identify the nucleus and cytoplasm, respectively. The black boxes identify the neurons enlarged in the insets. Scale bars: 100 μm. g-h. The frequency of disrupted NE staining for both SUN1 (g) and SUN2 (h) was quantified blindly in at least two sections from each patient’s tissue. A significant increase in the percentage of cells with disrupted staining was observed in both sALS and C9-ALS spinal cords compared to controls. A significant higher frequency of disruption in C9-ALS neurons was detected for SUN2. A similar trend was observed for SUN1, but it did not reach statistical significance. Each dot represents the mean of at least two sections for each case, horizontal lines show mean and standard deviation (one-way ANOVA with Tukey post hoc test, n = 5, 5, and 3, *p < 0.05, **p < 0.01, ***p < 0.001, ns = not significant)