Fig. 1
From: Neuronal network dysfunction in a model for Kleefstra syndrome mediated by enhanced NMDAR signaling
Generation and characterization of iPS cell-derived neurons from KS patients. a KS and control iPS lines used in this study. b Western blot and quantification of EHMT1 protein levels in iPS cells, n = 6–7 for each condition. c Schematic presentation of the differentiation protocol, including representative images of control iNeurons immunostained for MAP2 during development (scale bar 10 µm). d Representative somatodendritic reconstructions of control and KS iNeurons (scale bar 50 µm). e Representative images of control and KS iNeurons stained for MAP2 (red) and synapsin 1/2 (green) at DIV 21 (scale bar 5 µm) and quantification of synapsin puncta, n = 25 for C1; n = 13 for C2; n = 15 for CMOS; n = 17 for KS1; n = 11 for KS2; n = 15 for KSMOS. f Representative example traces of mEPSCs from control and KS iNeurons at DIV 21. Quantification of the frequency and amplitude of mEPSCs in control (C1, CMOS, and CCRISPR) and KS (KS1, KSMOS, and KSCRISPR) iNeurons (i.e., pooled value for control and KS iNeurons), n = 30 for C, n = 29 for KS. Data represent means ± SEM. **P < 0.005, ***P < 0.0005, ****P < 0.0001, one-way ANOVA test and post hoc Bonferroni correction was performed between controls and KS iNeurons and Mann–Whitney test was performed between two groups. Source data is available as a Source Data file
