Fig. 4

Insoluble TDP-43 is elevated in motor neuron-differentiated ALS-patient derived iPSCs upon FICZ treatment. Immunoblots of RIPA insoluble material a and total lysates d from motor neurons differentiated from an ALS-affected patient carrying a G298S mutation in TARDBP. Both a short exposure of the monomeric TDP-43 band and a longer exposure of the full blot are shown in a. Densitometric analysis of TDP-43 b and TDP-35 c from RIPA insoluble immunoblots indicates that AHR agonism by 0.1 μM FICZ treatment results in increased pathological species of TDP-43. Further, co-treatment with the AHR antagonist CB7993113 (5 μM) prevents the increase in RIPA insoluble TDP-43 and TDP-35 triggered by FICZ. N = 3; mean ± SEM, ANOVA w/Tukey’s; * P < 0.05, ** P < 0.01