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. 2021 Apr 30;4:509. doi: 10.1038/s42003-021-02034-z

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© The Author(s) 2021

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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Fig. 5 — a Experimental setup, mice were evaluated with behavioral tests from day 70 (baseline) and tested weekly until day 150. b Time point for onset of disease defined as visible tremor in hindlimbs, log-rank survival analysis. c Neuroscore was assessed once a week. Mean neuroscore ± SEM. d Hangwire test was performed once a week. Mean latency to fall of the grid ± SEM. e Grip strength was measured once a week from day 70. Data are presented as mean normalized strength ± SEM. f Mean body weight expressed in grams ± SEM. g Cylinder test was performed at day 70, 100, and 130. Mean rears ± SEM. One-way ANOVA with Tukey post hoc test. h Y-maze test was conducted at day 70, 100, and 130. Mean number of entries ± SEM. One-way ANOVA with Tukey post hoc test i Y-maze test was conducted at day 70, 100, and 130. Mean spontaneous alternation percentage ± SEM. One-way ANOVA with Tukey post hoc test. j Survival analysis for females, survival was defined as neuroscore below 4, log-rank test. k Mean neuroscore for SOD1 mice evaluated from day 70 and until day 132 ± SEM. l Survival analysis for females, survival was defined as neuroscore below 4, log-rank test. Animals were tested weekly from day 70 of age until day 148. N = 5–10, except for tremor onset (n = 12–16) and survival analyses (n = 8–10 for females, n = 10–25). Data are representative of two independent animal experiments for females from day 70 and until day 150 and one for day 70 and until day 132. If nothing else is noted, data was analyzed using repeated measure two-way ANOVA followed by Tukey post hoc test. *Significant differences between SOD1^Cpt1a/Cpt1a and SOD1, #significant differences between SOD1^Wt/Cpt1a and SOD1 in behavioral tests. *p ≤ 0.05; **p ≤ 0.01; ***p ≤ 0.001, ****p ≤ 0.0001. SOD1 = SOD1 G93A genotype, SOD1^Wt/Cpt1a = SOD1 G93A mice with heterozygote Cpt1a P479L mutation, SOD1^Cpt1a/Cpt1a = SOD1 G93A mice with homozygote Cpt1a P479L mutation. Mouse figure used in experimental setup figures were obtained from; https://smart.servier.com/ and used according to the Creative Commons Attribution 3.0 Unsupported License.