Supplementary Figure 14: PLX3397 treatment itself does not affect transgene expression, suppression or overall motor function.

(a) Though the hTDP-43 levels are different at the protein level, qPCR analysis reveals that the control and PLX3397-treated animals both have similar low hTDP-43 mRNA levels in rNLS8 mice maintained continuously on DOX (black bar, n = 3), off DOX for 6 weeks (white bar, n = 5), or 4 weeks off DOX + 2 weeks on and treated with PLX3397 (blue bar, n = 4) or control Nutella (grey bar, n = 4), Bars represent mean ± S.D. (b-e) Representative cryosections of lumbar SC from sham-treated (b,d) and PLX3397-treated (c,e) rNLS8 mice immunostained for IBA-1 (red) and LPL or Trem2 (blue) shows that surviving microglia in the PLX3397-treated lumbar SC still express LPL and Trem2. Staining was performed on tissue from n = 5 per treatment, with similar results. Scale bars = 100 μm (f-i). Treating nTg mice with the same PLX3397 regimen does not result in any obvious motor phenotype (f), change in maximum evoked CMAP from the gastrocnemius muscle (g), loss of MN in the SC (h), or axonal dieback from the TA muscle (i). Bars represent mean ± S.D., n = 4 mice.