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. 2021 Jan 7;16:1. doi: 10.1186/s13024-020-00420-5

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© The Author(s) 2021

Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

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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)