Fig. 8

IMS-088 treatment reversed the neuronal translational defects associated with TDP-43 proteinopathy. a NFL-rRFP;hTDP-43^A315T mice treated with IMS-088 or vehicle for 8 weeks and the mRNA bound to neuronal ribosome complex were analyzed with the Affymetrix 2.0 ST chip at 12 months of age. b Volcano plot of proteomic results of peptide isolated from brain neuronal ribosomal complex of NFL-rRFP;hTDP-43^A315T mice treated with IMS-088 or vehicle were analyzed with the mass spectroscopic methods at 12 months of age. Experiments were conducted in three biological replicates (n = 6 mice/condition). c GO functional analysis of isolated peptide from brain neurons of NFL-rRFP;hTDP-43^A315T mice treated with IMS-088 or vehicle revealed IMS-088 rescued the neuronal cytoskeletal deregulation in mhTDP-43 mice. Levels of (d) mRNA and (e) peptide detected for neuronal cytoskeletal proteins nefl, nefm and α-internexin (Statistical analysis used was p-value limma test; Data represents mean ± sem. ** p < 0.01). f Immunoblot and graph represent levels of α-internexin and NFL in the mouse brain of different groups (n = 3; 1-way ANOVA; Bonferroni’s multiple comparison test, * p < 0.05)