Fig. 5: Differentiation of SCA3 and isogenic control SCA3-NSCs into cerebral cortical neurons. | Translational Psychiatry

Fig. 5: Differentiation of SCA3 and isogenic control SCA3-NSCs into cerebral cortical neurons.

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

Fig. 5

a–c Immunofluorescence was used to detect the expression of surface markers and synaptic proteins in each group. Further differentiation resulted in neurons expressing TUJ1, MAP2, GABA, and GFAP. e and f The percentage of corresponding neuron markers were counted. Similar results were acquired with each cell line in our study (P > 0.05). Scale bar (TUJ1): 50 µm. Scale bar (MAP2, GABA, and GFAP): 25 µm. d, g Representative images of neuronal cells stained with SYP1 and PSD95 on days 35–50, there was no difference in the expression of SYP1/PSD95 in each group (P > 0.05). Scale bars 25 µm. h IC2 positive polyQ aggregates were detected in SCA3 neurons compared with other groups on day 50 (aggregates indicated in enlarged images with the arrow). Scale bar: 100 µm. b, d, f, h All data were presented as mean ± SD. The data as determined by one-way ANOVA and Bonferroni post hoc test. n = (6, 6, 6, 6, 6, 6) images for TUJ1, MAP2, GABA, GFAP, SYP1, and PSD95 neuronal markers expression. Ctrl-NCs: healthy control neurons. SCA3-NCs: SCA3 patient-derived neurons. SCA3-C3-NCs and SCA3-C12-NCs: corrected SCA3 neurons.

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