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. 2016 Jul;17(1):140–147. doi: 10.1016/j.scr.2016.06.003

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© 2016 The Authors. Published by Elsevier B.V.

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

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Fig. 5 — Functional properties of ALS iPSC-derived skeletal muscle cells.

(A) Dose-response relation of ACh-evoked currents in skeletal muscle cells differentiated as described in Fig. 4 from FUS^R514S/WT-MyoD (light gray bars, n = 7; §, p < 0.05 vs 3 μM; §§, p < 0.05 vs 3 μM and 30 μM; OneWay ANOVA) and from TDP-43^A382T/A382T-MyoD (dark gray bars, n = 9; *, p < 0.05 vs WT I-MyoD cells, TwoWay ANOVA) iPSCs, compared to those recorded in WT I cells (white bars, n = 11; #, p < 0.05 vs 3 μM; #, p < 0.05 vs 3 μM and 30 μM; OneWay ANOVA). (B) Average fluorescence response to ACh (300 μM) in Fura-2-loaded iPSC-FUS^R514S/WT-MyoD (light gray trace; n = 18 cells) and iPSC-TDP-43^A382T/A382T-MyoD (dark gray trace; n = 13 cells) cells.