Fig. 5

Membrane repair and fusion index using oprozomib, ixazomib and EB1089 treatment. a Representative images of PI-positive nuclei and MyHC positive myotubes after SDS treatment at 90 s and at 10 min. The first 2 rows correspond to healthy myotubes and the last 2 rows correspond to dysf mut/mut myotubes treated with oprozomib. b Quantification of IP-positive nuclei in myotubes from healthy controls and dysf mut/mut myotubes treated with EB1089, oprozomib, and the combination of oprozomib 10 nM with EB1089at at 24 h. c Quantification of IP-positive nuclei in myotubes from healthy controls and dysf mut/mut myotubes treated with EB1089, ixazomib, and the combination of ixazomib 100 nM with EB1089at at 8 h. Differences were not observed with any treatment. d Quantification of fusion index showed a statistically significant decrease of MyHC positive myotubes compared to healthy myotubes. e Fusion index in dysf mut/mut myotubes treated with EB1089, oprozomib at 10 nM and the combination of oprozomib 10 nM with EB1089 at 8 h did not show any difference compared to non-treated myotubes. f Fusion index in dysf mut/mut myotubes treated with EB1089, ixazomib at 100 nM and the combination of ixazomib 100 nM with EB1089 at 24 h did not show any difference compared to non-treated myotubes. Data are represented as mean of 3 replicates ± standard deviation. Results were statistically analyzed using Mann-Whitney test to compare two groups and one-way Anova followed by Tukey post hoc test for multiple comparisons. Statistical significance was set at p < 0.05. *p < 0.05; **p < 0.01; ***p < 0.0001