Figure 1.

Neuronal network development for LH1-3, and HH1-3 neuronal networks

(A) Patient-derived fibroblasts were reprogrammed to iPSCs, generating low (0%) and high (60%–65%) heteroplasmy clones. These were differentiated into excitatory neurons, co-cultured with rat astrocytes, on MEAs recorded for a 10-min period at DIV30, -DIV37, and -DIV44.

(B) Representative image of iPSC-derived neurons and co-cultured rat astrocytes at 1:1 ratio, stained for MAP2 (green), GFAP (red), and Hoechst (blue) at DIV30 (scale bar, 30 μm).

(C–E) Representative raster plots of MEA recordings, as well as the quantification of the MFR, the PRS, and the NBR, for (C) LH1 (n = 27) and HH1 (n = 27), (D) LH2 (n = 16) and HH2 (n = 14), and (E) LH3 (n = 23) and HH3 (n = 21). Data represent means ± SEM. ^∗p < 0.05, ^∗∗p < 0.01, ^∗∗∗p < 0.001, ^∗∗∗∗p < 0.0001, using restricted maximum likelihood model, with Holm-Sidak's correction for multiple comparisons.

See also Figure S1.