Effect of Z-FA-FMK on the expression of SMN proteins in SMA patient iPSCs and iPSC-derived motor neurons. Effect of Z-FA-FMK on the expression of SMN proteins.

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Effect of Z-FA-FMK on the expression of SMN proteins in SMA patient iPSCs and iPSC-derived motor neurons. Effect of Z-FA-FMK on the expression of SMN proteins in SMA patient iPSCs and iPSC-derived motor neurons. (A) Phase images showing representative stages during motor neuron differentiation from iPSCs (day 0). NE, neuroepithelial cells (1-wk); MN, motor neuron (3-wk). Scale bars, 50 μm. (B) Western blot analysis of the SMN protein expression in iPSCs. (C) Quantification revealed a dose-dependent increase of SMN proteins in iPSCs derived from a SMA type I patient after Z-FA-FMK treatment. Data were presented as mean ± SEM, n = 3. (D) Western blot analysis revealed an increased expression of SMN proteins after Z-FA-FMK treatment at day-24 motor neuron stage. (E) The activity of caspase 3/7 was significantly increased in motor neuron long-term cultures (6-wk) derived from SMA iPSCs, and this increase was significantly inhibited by the application of Z-FA-FMK. Mean ± SEM, n = 5. **P < 0.01 as compared with the WT group, ##P < 0.01 as compared with the SMA+DMSO group by two-tailed t test. The dose-dependent effect of Z-FA-FMK (C) in SMA iPSCs was confirmed using linear regression (P = 0.0008). Yiran Wang et al. LSA 2019;2:e201800268 © 2019 Wang et al.