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. 2020 Aug 31;10:14302. doi: 10.1038/s41598-020-70510-3

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© The Author(s) 2020

Open Access This 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 license, and indicate if changes were made. The images or other third party material in this article are included in the article’s Creative Commons license, unless indicated otherwise in a credit line to the material. If material is not included in the article’s Creative Commons license 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 license, visit http://creativecommons.org/licenses/by/4.0/.

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Mitochondria fsunctional assessment. (A) TMRE assay measuring fluorescence intensity as a measure of mitochondrial membrane potential at day 12 of differentiation. SOD1-mutant muscle exhibit decreased mitochondrial membrane potential compared to WT Ctrl A. Error bars: SEM *p < 0.1; **p < 0.01; ***p < 0.001 by Student T-test, 2 tails, unequal variance. (B, C) Mitochondrial metabolic rate assessment of SOD1 muscle for TCA cycle intermediates and glucose-derived substrates. (D–F) Mitochondrial metabolic rate assessment of SOD1 muscle for amino acid-, fatty acid- and ketone-derived substrates respectively.