Fig 2: Ibuprofen improves phenotypes in cells harboring the mutants. (A) Cells harboring POLR3B (R550X) were allowed to differentiate in the presence or absence of ibuprofen for 5 days. Some cells in the upper images are surrounded by white dotted lines. The square fields indicated by the dotted lines in the upper panels are magnified in the respective lower panels. (B) Cells with widespread membranes were statistically assessed (** p < 0.01; n = 5 fields (50 cells in total)). (C) The lysates of the respective cells were immunoblotted with an antibody against PLP1, CNPase, Sox10, and actin. (D) Their expression levels are shown statistically compared to their respective controls (** p < 0.01; n = 3 blots).

Fig 3: Oligodendrocyte pathology in TDP-43 mice.(A) Age-dependent and progressively increased activated caspase 3 signals are associated with oligodendrocytes (arrows) in the spinal cord of Tg mice. (B) Reduced myelin protein levels in the Tg mice with paralysis. M = month, CNPase = 2’,3’-cyclic-nucleotide 3’-phosphodiesterase, MBP = myelin basic protein, MCT1 = monocarboxylate transporter 1. (C) A reduced staining intensity of CNPase in the paralyzed Tg mice compared with the nTg mice.

Fig 4: Ibuprofen does not have significant effects on phenotypes in cells harboring the wild type POLR3B. (A) Cells harboring the wild type were allowed to differentiate in the presence or absence of ibuprofen for 5 days. Some cells in the upper images are surrounded by white dotted lines. The square fields indicated by the dotted lines in the upper panels are magnified in the respective lower panels. (B) Cells with widespread membranes were statistically assessed (n = 5 fields (50 cells in total)). (C) The lysates of the respective cells were immunoblotted with an antibody against PLP1, CNPase, Sox10, and actin. (D) Their expression levels are shown statistically compared to their respective controls (n = 3 blots).