(A) C9-ALS iPSNs treated with DMSO, GSK or ISRIB were stained with Ran (red), MAP2 (green), and DAPI (blue). (B) Control or C9-ALS iPSNs treated with scrambled or Ataxin-2 ASOs were stained with Ran (red), MAP2 (green), and DAPI (blue). Bottom right: ASO-treated C9-ALS iPSNs immunoblotted for Ataxin-2 and β-Actin. (C) Fly salivary glands stained with GFP and DAPI. N: nuclear; W: whole cell. (D) Fly eye degeneration. (E) Flight assay. n numbers in the graph. ns: not significant; *: p<0.05; **: p<0.01; ***: p<0.001; ****: p<0.0001. Data are represented as mean ± SEM.