Fig. 5

A) Characterization of the neurons to validate their excitatory nature: Mean firing rate of control (19-2) neurons were recorded using MEA during four consecutive readings. Recordings were done after treatment with neurotoxins. Untreated; PTX: Picrotoxin; CNQX: 6-Cyano-7-nitroquinoxaline-2, 3-dione; TTX: Tetrodotoxin (N = 8; mean ± SEM; one-way ANOVA, Sidak's multiple comparisons test). B) and C) Representative Raster plots of neuronal network activity of representative wells of control vs DDX53-3TC. Each row represents an individual microelectrode of the MEA device. Each black bar represents one spike and each row of black bars (spike train) represents spiking activity of a neuron, over a period of 100 s, blue bars represents the burst. Multi-electrode array analysis of DDX53-3TC neurons and respective isogenic controls. D) Weighted mean firing rate of control neurons, and DDX53 mutant neurons (19-2-DDX53-3TC) from 3 to 6 weeks (N: controls for week 3 = 8; week 4 = 16; week 5 = 8; week 6 = 16. N: 19-2-DDX53-3TC for week 3 = 11; week 4 = 15; week 5 = 8; week 6 = 15; mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test). E) Burst frequency of control neurons, and DDX53 mutant neurons (19-2-DDX53-3TC) from 4 to 6 weeks (N: controls for week 4 = 8; week 5 = 8; week 6 = 16. N: 19-2-DDX53-3TC for week 4 = 8; week 5 = 8; week 6 = 15; mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test). F) Network burst frequency of control neurons, and DDX53 mutant neurons (19-2-DDX53-3TC) from 5 & 6 weeks (N: controls for week 5 = 8; week 6 = 16. N: 19-2-DDX53-3TC for week 5 = 8; week 6 = 16; mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test). G) Weighted mean firing rate of control neurons, and DDX53 mutant neurons (50B-DDX53-3TC) from 3–6 weeks (N: controls for week 3 = 18; week 4 = 29; week 5 = 24; week 6 = 26. N: 50B-DDX53-3TC for week 3 = 16; week 4 = 25; week 5 = 24; week 6 = 23; mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test). H) Burst frequency of control neurons, and DDX53 mutant neurons (50B-DDX53-3TC) from 4–6 weeks (N: controls for week 4 = 25; week 5 = 24; week 6 = 23. N: 50B-DDX53-3TC for week 4 = 24; week 5 = 23; week 6 = 21 mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test). I) Network burst frequency of control neurons, and DDX53 mutant neurons (50B-DDX53-3TC) from 4 to 6 weeks (N: controls for week 4 = 8; week 5 = 24; week 6 = 17. N: 50B-DDX53-3TC for week 4 = 6; week 5 = 23; week 6 = 21 mean ± SEM, two-way ANOVA, Sidak's multiple comparisons test)