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. 2017 Oct 27;7:14275. doi: 10.1038/s41598-017-14290-3

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© The Author(s) 2017

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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Treatment with ACVR2B/Fc improves muscle function in R6/2 mice. (A–D) Contractile dysfunction of the R6/2 EDL muscles was assessed by measuring twitch tension: the time to peak and half relaxation time and this was corrected by ACVR2B/Fc treatment in both EDL (A,C) and TA (B,D) muscles. (E,F) The maximum tetanic force in mice treated with ACVR2B/Fc and vehicle. The maximum EDL (E) and TA (F) forces were decreased in R6/2 mice and completely rescued in mice treated with ACVR2B/Fc. (G) Examples of motor unit traces and the quantification of functional motor units. The number of functional motor units was reduced in R6/2 EDL muscles and this was restored in treated mice. Statistical analysis was two-way ANOVA with post-hoc Bonferroni correction (see Table S8 for main effects and Table S9 for multiple comparisons). Statistically significant differences between vehicle treated WT and vehicle treated R6/2: ^# p < 0.05, ^### p < 0.001; statistically significant differences between ACVR2B/Fc treated R6/2 and vehicle treated R6/2: *p < 0.05; **p < 0.01; ***p < 0.001. n = 11 WT vehicle (EDL), n = 12 WT vehicle (TA), n = 8 WT ACVR2B/Fc (EDL and TA), n = 14 R6/2 vehicle (EDL and TA), n = 12 R6/2 ACVR2B/Fc (EDL and TA). All data presented as means ± SEM. The baseline WT and R6/2 vehicle treated phenotype data were previously published¹⁰.