Fig. 3
Excitability of MNs derived from mutant C9ORF72 patient and control iPSCs. a Evoked firing types observed in iPSC-derived MNs. MNs displayed either multiple, single, or no action potentials upon current stimulation. b Percentage of cells showing each category of firing did not differ between MNs derived from control (Con-1, n = 31; Con-2, n = 53), C9ORF72 mutant (C9-1, n = 17; C9-2, n = 32; C9-3, n = 73), and gene-edited (C9-1∆, n = 37; C9-2∆,n = 34; C9-3∆, n = 84) iPSC lines. c Raw traces showing repetitive action potential firing (>5 action potentials) in response to current injection in MNs derived from control, C9ORF72 mutant, and gene-edited iPSC lines. d F–I relationships generated from repetitively firing MNs reveal no significant differences between MNs derived from control (Con-1, n = 8; Con-2, n = 23), C9ORF72 mutant (C9-1, n = 3; C9-2, n = 5; C9-3, n = 22), and gene-edited (C9-1∆, n = 3; C9-2∆, n = 4; C9-3∆, n = 39) iPSC lines (mean ± s.e.m. values are plotted in the graph). Calibration bars: a: 200 ms, 20 mV; c: 400 ms, 20 mV
