Fig. 5.

Scn2a deletion in dorsal telencephalic excitatory but not global inhibitory neurons triggered SWDs in mice. a Survival rates at P2.5 of Scn2a^fl/fl (N = 7), Scn2a^fl/fl/Emx1-Cre (N = 6) and Scn2a^fl/fl/Vgat-Cre mice (N = 5). All Scn2a^fl/fl/Emx1-Cre and all but one Scn2a^fl/fl/Vgat-Cre mice died before P2.5. One Scn2a^fl/fl/Vgat-Cre survivor died at P8.5. b Survival curves during P3–30 of Scn2a^fl/+/Emx1-Cre (N = 39), Scn2a^fl/+/Vgat-Cre (N = 30) and Scn2a^fl/+ mice (N = 95). About 30% of Scn2a^fl/+/Vgat-Cre mice suffered premature death between P16 and P25. c Representative ECoG/EMG traces in Scn2a^fl/+/Emx1-Cre mice. SWDs during waking were often associated with behavioral arrest (right). Black arrowheads indicate the onsets of SWDs. Gray arrowheads indicate the onset and end of behavioral arrest. d, e Frequencies of SWDs during 24 h ECoG recordings in Scn2a^fl/+/Emx1-Cre (N = 5) and littermate controls (Cntl) (2 Scn2a^fl/+, 3 Scn2a^+/+/Emx1-Cre, N = 5), Scn2a^fl/+/Vgat-Cre (N = 4) and littermate Cntl (2 Scn2a^fl/+, 1 Scn2a^+/+/Vgat-Cre, N = 3), and Scn2a^RX/+ mice (N = 7). All recorded mice were over 8 weeks of age. f Ethosuximide (33.3 mg mL⁻¹ in saline, 200 mg per kg, i.p.) efficiently suppressed SWDs in Scn2a^fl/+/Emx1-Cre mice (N = 6). g, h LFP recordings from 3–6-month-old Scn2a^fl/+/Emx1-Cre (N = 4) and littermate Cntl (2 Scn2a^+/+, 2 Scn2a^fl/+, 3 Scn2a^+/+/Emx1-Cre, N = 7). g Representative ECoG/EMG/LFPs traces in Scn2a^fl/+/Emx1-Cre mice. h Epileptiform discharges were predominantly detected in medial prefrontal cortex and caudate putamen of Scn2a^fl/+/Emx1-Cre mice (Mann–Whitney test, ECoG, somatosensory cortex: U = 0, *P = 0.0286; LFP, medial prefrontal cortex: U = 0, *P = 0.0286; visual cortex: U = 6, P > 0.999; basolateral amygdala: U = 0, *P = 0.0268; hippocampus CA1: U = 4, P = 0.4286; caudate putamen: U = 0, *P = 0.0268; ventroposterior thalamus: U = 0, *P = 0.0286). Data represent means ± SEM, *P < 0.05, **P < 0.01. Scale bars: (c, g) vertical 0.5 mV; horizontal 1 s