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. 2021 Jul 15;13(8):e13610. doi: 10.15252/emmm.202013610

Figure EV4. Spinocerebellar ataxia type 12 forebrain organoids presented normal cortical development.

Figure EV4

PBMCs were isolated from SCAR12 (G372R mutation) patients and from their healthy parents were reprogrammed into iPSCs, and subsequently were differentiated into forebrain organoids.
  1. Week 4 forebrain organoids (FOs) of the healthy, heterozygote father (WPM F2) and mother (WPM M3), and their sick homozygotes daughter (WPM D1) and son (WPM S1) stained for WWOX, β3‐tubulin, and SOX2 expression (WPM F2: n = 2 from 1 batch; WPM M3: n = 2 from 1 batch; WPM D1: n = 2 from 1 batch; and WPM S1: n = 2 from 1 batch). Scale = 50 µm (left), 20 µm (right).
  2. qPCR for the assessment of the expression of different neural markers in week 20 FOs.
  3. qPCR for the measurement of expression levels of cortical layer markers in week 20 FOs.
  4. Expression level of Wnt pathway‐related genes at mRNA levels quantified using qPCR in week 20 FOs.

Data information: B‐D: Data are represented as mean ± SEM. Statistical significance was determined using one‐way ANOVA with Tukey’s multiple comparisons test (WPM F2: n = 4 from 1 batch; WPM M3: n = 3 from 1 batch; WPM D1: n = 4 from 1 batch; and WPM S1: n = 3 from 1 batch). *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001, and ****P ≤ 0.0001.