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. 2019 Aug 7;13:361. doi: 10.3389/fncel.2019.00361

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Copyright © 2019 Guo, Wang, Ma, Cui, Yu, Liu, Xue, Zhu, Cao, Li, Tang and Chen.

This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.

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USH2A mutation neural retina (NR) organoids have abnormal organoid induction and increased apoptosis. (A) A schematic representation of patient-specific iPSC differentiation along the NR organoid lineage. Brightfield images of developing NR over time (B) and USH2A mutation iPSCs generated NR at a slower pace (C), less efficiently (D), and with a smaller diameter (E) versus controls. (F) qPCR analysis revealed mRNA levels of neuron apoptosis cell specific markers CASP3, MSH2, ADARB1, and HIF1A (GAPDH gene as a control). (G) Immunostaining of retinal organoids showing the expression of cell-proliferation marker Ki67. (H) Neuron apoptotic process related DEGs at day 34 were normalized by Z-scores. Red represents upregulated expression. Blue represents downregulated expression, Bar is represents Z-score. (n = 3 independent experiments; each experiment need 2–4 organoids; scale bar = 200 μm).