Figure 3.

E1^MOv11 transient transfection results in a significant improvement in full-length SMN expression in induced pluripotent stem cell (iPSC) derived motor neuron cultures. (a) An increase in full-length SMN transcript after E1^MOv11 treatment as measured by RT-PCR. (b) Quantification full-length SMN transcripts from iPSC derived motor neurons. Two concentrations of E1^MOv11 resulted in significant increase of full-length SMN mRNA expression and a decrease in SMN▵7 mRNA when compared with sham-transfected cultures. *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001. (c) Representative images of iPSC derived motor neuron cultures. Astrocytes within the cultures were stained for GFAP (red). Cytoplasmic expression and nuclear localization of SMN (green) was increased with E1^MOv11 treatment. Nuclei were labeled with Hoechst (blue). Scale bar = 10 μm. RT-PCR, reverse transcriptase-polymerase chain reaction; SMN, survival motor neuron; GFAP, glial fibrillary acidic protein.