Skip to main content
. Author manuscript; available in PMC: 2018 Aug 1.
Published in final edited form as: Nat Neurosci. 2018 Jan 8;21(2):228–239. doi: 10.1038/s41593-017-0047-3

Figure 8. Pharmacological inhibition of TDP-43 toxicity rescues nucleocytoplasmic transport function caused by TDP-43 pathology.

Figure 8

a, Overexpression of TDP-CTF or mutant TDP-43Q331K increases cell death in transfected cortical neurons compared to GFP. Treatment of 50 nM KPT-335 for 24 hours reduced cell death in both TDP-CTF and TDP-43 Q331K. b, Abnormal nuclear morphology with lamin B staining in neurons expressing TDP-CTF or TDP-43Q331K is rescued via the treatment of 50 nM KPT-335. c, Locomotion defects in larva expressing human TDP-43WT and TDP-43G298S is ameliorated by treatment with 1 μM but not 5 μM PT-335 or KPT-276. Graphs represent quartiles (boxes) and range (whiskers). Five independent experiments for a (circles represent each independent experiment; * P < 0.05, ** P < 0.01, *** P < 0.001, two-way ANOVA), four independent experiments for b (circles represent each independent experiment; * P < 0.05, *** P < 0.001, two-way ANOVA) and c (circles represent n = 30 animals; ** P < 0.01, *** P < 0.001, two-way ANOVA). Bonferroni’s post hoc test. Full statistical details are provided in Supplementary Table 4.