Fig. 1

Altered network function in C9ORF72^RE-derived cortical neurons. a Left, Representative MEA recordings from control (blue), C9ORF72^RE- (black) and respective C9ORF72^RE gene-edited- (red) derived neuronal cultures each showing traces from four electrodes from single arrays. Note the difference in the network burst properties in C9ORF72^RE-derived cultures. Scale bar, 50 μV, 30 s. Right, Mean (± s.e.m.) data for control (Con), C9ORF72^RE- (C9) and respective C9ORF72^RE gene-edited- (C9-Δ) derived neurons for interburst and network burst lengths (Con, N = 9; C9, N = 10; C9-Δ, N = 9) and intra-network burst spiking frequencies (Con, N = 7; C9, N = 6; C9-Δ, N = 8). Data are pooled for C9 and C9-Δ – individual pair comparisons are presented in Supplementary Figure 4A. b Left, Representative current-clamp recordings from control, C9ORF72^RE- and respective C9ORF72^RE gene-edited-derived neurons (cells were held at − 74 mV; scale bar, 20 mV, 30 s). Right, Mean (± s.e.m.) data for control (Con), C9ORF72^RE- (C9) and respective C9ORF72^RE gene-edited- (C9-Δ) derived neurons for interburst lengths, burst lengths and intraburst spiking frequencies (Con, n = 10, N = 4; C9, n = 16, N = 4; C9-Δ, n = 13, N = 4). Data are pooled for C9 and C9-Δ – individual pair comparisons are presented in Supplementary Figure 4B. Note that differences in burst properties between MEA and patch-clamp experiments are likely to reflect differing resting membrane potentials and recording temperatures at which experiments are conducted and/or the alternate plating order of neurons and astrocytes for each respective approach. Significance determined by one-way ANOVA with Bonferroni’s post hoc test