Fig. 6

Synaptic defects in MERRF neurons harboring the m.8344A > G mutation. A The protein expression levels of presynaptic vesicular proteins and excitatory glutamate receptors in normal and MERRF neurons. B Quantitative data of the synaptic vesicular proteins, synaptophysin and vGLUT2. C Quantitative data of the excitatory receptors, AMPARs and NMDARA. Quantification of the proteins in Western blot was normalized with β-actin. All data are presented as the fold change of that in N neurons. D Representative image of spike histograms showing the spontaneous network bursting in N neurons and MERRF neurons from 2-min recording plotted over all electrodes. Temporal changes in E the number of network bursts, F network burst duration, and G the number of spikes per network burst during 10 weeks of differentiation. Data are presented as the average of 3–4 replicate wells of each neuron group in the experiment. H Quantitative parameters in N neurons and MERRF neurons at 7 weeks of maturation. Data are presented as mean ± SEM, n = 3. *p < 0.05; **p < 0.01; ***p < 0.001