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. 2020 Oct 31;8:177. doi: 10.1186/s40478-020-01060-y

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© The Author(s) 2020

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. 6 — G4C2 RNA foci modulated by MATR3 in C9-ALS patient iPSC-MNs. a Representative confocal images of control (left) and C9-ALS patient (right) iPSC-MNs, indicated by MAP2 (red), transfected with scrambled siRNA or MATR3 siRNAs. FISH-IF was performed to stain nuclear G4C2 RNA foci (green; yellow arrowheads) in these neurons. b, c Quantification of the percentage of neurons that are G4C2 foci-positive (b), and number of G4C2 foci per neuron (c) in iPSC-MNs from one control (Ctrl #1) and two independent C9-ALS (C9-ALS #1, C9-ALS #2). Knocking down MATR3 significantly reduced the percentage of neurons that form G4C2 foci (b) and also reduced number of G4C2 foci per neuron (c) compared to scrambled-control in both C9-ALS iPSC-MNs (Kruskal–Wallis test). n = 24-50 neurons Each data point (circle) represents data from neurons pooled from 3 independent differentiations. Error bars indicate S.E.M. ****p value < 0.0001