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. 2023 Aug 17;18(9):1870–1883. doi: 10.1016/j.stemcr.2023.07.004

Figure 6.

Figure 6

The iPSC-derived sMNs from sALS patients exhibit an increased spontaneous firing

(A) Representative images of iPSC-derived day 28 sMNs on a 48-well MEA plate with 16 microelectrodes/well. Scale bar, 100 μM.

(B) The MEA recording plot showing typical network firing patterns detected from iPSC-derived sMNs on day 28.

(C) The MEA recordings were carried out on iPSC-derived sMNs from day 18 to day 48. The control data were averaged from three lines of ALSH84C5, ALSH47C1, and LQTH002C4 of three independent donors, and patient data from four lines (ALS69C4, ALS53C5, ALS57C1, and ALS57C4) of three sALS patients.

(D–F) sMNs generated from patients ALS53, ALS57, and ALS69 had a significantly higher AP rate (weighted mean firing rate) (D), burst frequency (E), and network burst frequency (F) at the late stages of differentiation than the sMNs from the control group. The total MEA wells examined were n = 22 wells from N = 3 control iPSC lines, and n = 15 wells from N = 4 patient iPSC lines. The data were presented as mean ± SEM. See also Table S3.