Fig. 1

Cellular age increases the frequency of nuclear lamina disruptions independent of the presence of the C9orf72 HRE. a Immunostaining and confocal imaging for Lamin B1 in iPSNs at day 32 of differentiation. Genotype as indicated on left, antibody and iPSC passage number as indicated on top. b, c Quantification of nuclear sphericity (b) and nuclear volume (c). n = 8 control and 8 C9orf72 iPSC lines, at least 100 Map2 + neurons per line and iPSC passage. Two-way ANOVA Tukey’s multiple comparison test was used to calculate statistical significance. *p < 0.05, **p < 0.01. d Quantification of number of Lamin B1 invaginations per nucleus. n = 8 control and 8 C9orf72 iPSC lines, at least 100 Map2 + neurons per line and iPSC passage. Chi-square test was used to calculate statistical significance. ****p < 0.0001. Scale bar = 10 μm