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. 2019 Aug 6;9:11392. doi: 10.1038/s41598-019-47818-w

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© The Author(s) 2019

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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Local administration of ACE-083 in a Trembler^J mouse model of CMT causes focal hypertrophy of skeletal muscle, increases muscle force generation and improves ankle dorsiflexion torque. TrJ/Pmp22 mice (CMT mice) were given unilateral injections of ACE-083 (100 µg) or vehicle (PBS) in the TA muscle twice weekly for 4 weeks. (a) Weights of ACE-083-injected, vehicle-injected, and uninjected (contralateral) TA muscles. Data are means ± SEM (n = 10). ***P ≤ 0.001 vs. vehicle; ^###P ≤ 0.001 vs. uninjected (contralateral) TA by one-way ANOVA with Tukey’s adjustment. (n = 10–12). (b) Physiological cross-sectional area of ACE-083-injected, vehicle-injected, and uninjected (contralateral) TA muscles. Data are means ± SEM (n = 7–11). ***P ≤ 0.001 vs. vehicle; ^###P ≤ 0.001 vs. uninjected (contralateral) TA by one-way ANOVA with Tukey’s adjustment. (n = 7–11). (c) Photomicrographs of representative cross-sections stained with haematoxylin-eosin to demarcate individual fibers from ACE-083-injected and uninjected (contralateral) TA muscles. Scale bar, 100 µm. (d) Histograms of muscle fiber diameter in ACE-083-injected and uninjected (contralateral) TA muscles. Data represent 200 fibers per muscle (n = 10 mice). (e) Peak twitch force generated by ACE-083-injected or uninjected (contralateral) TA muscles as determined in situ. Data are means ± SEM (n = 5). ***P ≤ 0.001 vs. contralateral TA by unpaired t-test. (f) Peak tetanic force generated by ACE-083-injected or uninjected (contralateral) TA muscles as determined in situ. Data are means ± SEM (n = 5). ***P ≤ 0.001 vs. contralateral TA by unpaired t-test. (g) Ankle dorsiflexion torque determined in vivo under isometric conditions in ACE-083-injected or vehicle-injected CMT mice. Data are means ± SEM (n = 5). *P ≤ 0.05 vs. vehicle-injected mice. (h) Levels of Fbxo32 mRNA encoding muscle atrophy biomarker atrogin-1 in ACE-083-injected or uninjected (contralateral) TA muscles. Data are means ± SEM (n = 11). **P ≤ 0.001 vs. uninjected (contralateral) TA by Mann-Whitney U test.