Fig. 1
From: LINC complex alterations are a key feature of sporadic and familial ALS/FTD
LINC complex disruption in C9 iPSCs-derived iMNs. Representative images of SUN2 (a, grays) and Nesprin1 (e, Nesp1, grays) expression in C9 and isogenic control (CTRL) iMNs. Nuclei were identified by DAPI staining (blue), while MAP2 (red) and ISL1 (green) were used as neuronal and motoneuronal markers. The white boxes indicate the neurons enlarged in the panels on the right. Scale bars: 20 μm in main panels, 10 μm in zoomed-in images. The relative quantification of the nuclear mean fluorescence intensity (MFI) for both SUN2 (b) and Nesprin1 (f) shows a significant reduction in the abundance of each protein in C9 iMNs compared to isogenic controls (Mann-Whitney t test, n = 57 and 52 for CTRL and C9-ALS neurons respectively from 4 independent differentiations for SUN2, n = 64 and 40 for CTRL and C9 neurons respectively from 4 independent differentiations for Nesprin1, **** p < 0.0001). c-d. Representative western blot (WB) and quantification of SUN2 levels relative to GAPDH expression shows a significative reduction of total SUN2 levels in both C9 isogenic lines (24a and C52) compared to isogenic counterparts (n = 8 independent experiments for both C9 and controls; Student’s t test, **** p < 0.0001). For all, bars are mean and SEM, while violin plots show the distribution of the data with dashed lines indicating median and quartiles