RANBP17 Overexpression Restores Nucleocytoplasmic Transport and Ameliorates Neurodevelopment in Induced DYT1 Dystonia Motor Neurons

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Figure 8.
Figure 8.

Overexpression of RANBP17 ameliorates neurite outgrowth and neuron maturation in DYT1 neurons. A, Representative images of iMNs under indicated conditions at 21 dpi. The morphology was based on the ICC of TUBB3. Scale bar, 50 µm. B, The number of primary branches of iMNs at 21 dpi under indicated conditions. C, The number of secondary branches of iMNs at 21 dpi under indicated conditions. For B and C, N (neurons) = 57 for health, 52 for DYT1, 48 for DYT1 + GFP, and 53 for DYT1 + RANBP17 from four independent experiments. ns, no significant difference; **p < 0.01; ***p < 0.001, ****p < 0.0001. Student’s t tests. D, The relative neurite length of iMNs at 14 dpi under indicated conditions. N (neurons) = 127 for health, 112 for DYT1, 94 for DYT1 + GFP, and 93 for DYT1 + RANBP17 from four independent experiments. ns, no significant difference; **p < 0.01; ***p < 0.001, ****p < 0.0001. Student’s t tests. E, RT-PCR assay of genes related to neuron maturation in DYT1 iMNs with overexpression of RANBP17-GFP and GFP control at 14 and 21 dpi. N, three independent experiments. ns, no significant difference; *p < 0.05; **p < 0.01; ***p < 0.001. Student’s t tests.

  1. The Journal of Neuroscience vol. 44 no. 15
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