Fig. 6

NF aggregation and mitochondria density in WT neurons expressing G298S TDP43. a Schematic diagram showing TALEN-mediated insertion of G298S TDP43 or EGFP into the AAVS1 locus. b Western blots and relative expression of TDP43 (to GPDH) in MNs and non-MN derived from hESCs expressing G298S TDP43 or EGFP. c Phase contrast and corresponding GFP fluorescent images of MNs and non-MNs from the transgenic hESCs. d Immunofluorescent images of NF-H, NF-M, and NF-L in CHAT+ cells from TDP43- and EGFP-expressing hESCs. NF staining in the insets is magnified on the right panel. Arrows indicate NF inclusions in the cell body; arrowheads indicate NF inclusions in neurites. Scale bar = 50 μm. e Quantification of NF inclusion-containing cell bodies (c) and neurites (d) at days 4, 7, and 10 after plating neurons. f Mitochondria number decreased in TDP43-expressing MNs as compared to EGFP-expressing MNs. *p < 0.05. g Immunofluorescent images of NF-H, NF-M, and NF-L in non-MN cells from TDP43- and EGFP-expressing hESCs. Scale bar = 50 μm