Fig. 6: Electrophysiological characterization of cerebral cortical neurons differentiated from SCA3 and isogenic control SCA3-iPSCs. | Translational Psychiatry

Fig. 6: Electrophysiological characterization of cerebral cortical neurons differentiated from SCA3 and isogenic control SCA3-iPSCs.

From: CRISPR/Cas9 mediated gene correction ameliorates abnormal phenotypes in spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cells

Fig. 6

a Representative current-clamp recording of evoked action potentials from SCA3 neurons after injection of step currents (−20 to 50 pA). b Voltage clamp recordings sustained inward and outward currents, in response to depolarizing voltage steps (−80 to 80 mV) in Ctrl-NCs, SCA3-NCs, and SCA3-C3-NCs, respectively. c Quantification of inward and outward currents peak curves in Ctrl-NCs (n = 6), SCA3-NCs (n = 6), and SCA3-C3-NCs (n = 6). Similar results were detected among the groups (P > 0.05). d Representative traces of excitatory postsynaptic currents in Ctrl-NCs, SCA3-NCs, and SCA3-C3-NCs, respectively. e and f The amplitude and frequency of EPSC were similar in each group (P > 0.05). n = 7 in three groups. g–i The membrane capacitance, input resistance, and resting membrane potentials of Ctrl-NCs, SCA3-NCs, and SCA3-C3-NCs. No significant difference was shown in the three groups (P < 0.05). n = 7 in each group. All data were shown as mean ± SD. The data was determined by one-way ANOVA and Bonferroni post-hoc test. Ctrl-NCs: healthy control neurons. SCA3-NCs: SCA3 patient-derived neurons. SCA3-C3-NCs: corrected SCA3 neurons.

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