iPS-WT and iPS-SMA cells can generate cells in the neural lineage. a,b, iPS-WT and iPS-SMA cells generated nestin positive neural progenitor cells (green); c,d, Tuj1 positive neurons (green) and GFAP positive astrocytes (red); e,f, HB9 (green)/ChAT (red) double positive motor neurons, and g,h, SMI-32 (red) positive motor neurons at 4-6 weeks of differentiation i, At 8 weeks, punctate synapsin staining (green) on SMI-32 positive motor neurons (red) was identified on iPS-WT cells. j, However, only diffuse synapsin staining (green) was observed on SMI-32 positive motor neurons (red) in iPS-SMA cells. Arrowheads in i and j show punctate synapsin staining on SMI-32 negative cells. Nuclei are labeled with Hoechst nuclear dye (blue). k,l, iPS-SMA-derived motor neurons are significantly reduced in number and size at 6 weeks compared to iPS-WT cells (n=3 ANOVA, * p<0.05). All data are presented as mean ± s.e.m. Scale bar = 50μm (a-h), 25μm (i,j).