AdoMet reduces SG formation in iPSC-derived MNs and reduces TDP-43 accumulation in SGs. (A) Representative immunofluorescence images of AdoMet-treated or untreated control iPSC-derived MN lines and ALS-associated TDP-43 N352S and FUS R521G mutant iPSC-derived MN lines stained for TDP-43 and G3BP1 after treatment with 250 µM NaAsO2. (B) Box plots displaying quantification of the number of SGs/cell and total SG area from A. (C) Representative immunofluorescence images of AdoMet-treated or untreated control iPSC-derived MN lines and ALS-associated TDP-43 N352S and FUS R521G mutant iPSC-derived MN lines stained for TDP-43 and G3BP1 after treatment with 5 µg/ml puromycin. (D) Box plots displaying quantification of the number of SGs/cell and total SG area from A. (E) Representative immunofluorescence images of AdoMet-treated or nontreated WT iPSC-derived MN lines stained for Ataxin 2, G3BP1, and TDP-43 after treatment with 5 µg/ml puromycin. (F) Box plots displaying quantification of the total SG area as measured by G3BP1, Ataxin-2, and TDP-43 from A. Box plots in B, D, and F represent a compilation of three independent experiments. Scale bars in A, C, and E are 10 µm. Statistical significance in B, D, and F was determined by one-tailed unpaired Student’s t test (*, P < 0.05). For box plots, the ends of the box mark the 25th and 75th percentiles. The median is marked by a horizontal line inside the box. The whiskers mark the minimum and maximum measurements.