Figure 4.

Characterization of isogenic control and patient networks

(A) Representative raster plots showing 1 min of activity from three control (C₂, C₄, C₅)-MELAS (M_1-3) isogenic sets.

(B–E) Comparison of the MEA parameters (B) MFR, (C) PRS, (D) BD, and (E) NBR for each corresponding MELAS isogenic patient-control set (mean ± standard error of the mean).

(F) Burst shape and representative raw trace of a network burst from C₂ and M₁, C₄ and M₂, and C₅ and M₃ (sample size for C₂, n = 15, C₄, n = 23, C₅, n = 55, M₁, n = 22, M₂, n = 8, and M₃, n = 7, multiple t test on bins using the Holm-Sidak method, C₂ versus M₁, p < 0.001; C₅ versus M₃, p = 0.00021) (Table S4).

(G) PCA plots on seven7 MEA parameters for MELAS isogenic patient-control sets C2 and M1, C4 and M2, and C5 and M3 showing MEA parameters affected in MELAS.

(H) Representative raster plots showing 1 min of activity from two control (C_9-10)-KS patient (KS_3-4) isogenic sets.

(I–L) Comparison of the MEA parameters (I) BD, (J) NBR, (K) NBD, and (L) DT for each corresponding KS isogenic patient-control set (mean ± standard error of the mean).

(M) Burst shape and representative raw trace of a network burst from C₉ and KS₁, C₁₀ and KS₄ (sample size for C₉, n = 12, C₁₀, n = 17, KS₃, n = 16, and KS₄, n = 12, multiple t test on bins using the Holm-Sidak method) (Table S4).

(N) PCA plot on 12 MEA parameters for KS isogenic patient-control sets C₉ and KS₃ and C₁₀ and KS₄ showing MEA parameters affected in KS.

^∗p = 0.05, ^∗∗p = 0.01, ^∗∗∗p = 0.001. DIV, days in vitro; MFR, mean firing rate; PRS, percentage of random spikes; BR, mean burst rate; BD, mean burst duration; BSR, burst spike rate; IBI, inter-burst interval; NBR, network burst rate; NBD, network burst duration; NIBI, network burst IBI; CV_NIBI, coefficient of variation of all NIBI’s representing the regularity of the NB; RT, rise time; DT, decay time. All means, p values, and statistic tests used are reported in Table S4.