Fig. 4

LINC complex disruption in sALS and C9-ALS spinal cordpostmortem specimens. a-f. Spinal cord sections from control (a, d), sALS (b, e) and C9-ALS (c, f) patients were stained with antibodies specific for SUN1 (a-c) and Nesprin1 (d-f). Hematoxylin and eosin counterstains were used to identify the nucleus and cytoplasm, respectively. The black boxes identify the motor neurons enlarged in the insets. Scale bars: 100 μm. g-h. The frequency of disrupted NE staining for both SUN1 (g) and Nesprin1 (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. 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, ns = not significant)