Fig. 6: JQ1 treatment ameliorates pathological phenotypes in dystrophic muscle.

a JQ1 administration decreases NFkB p65-Ser536 phosphorylation in TA muscles from mdx mice (n = 5 for each experimental group). Lower panel: quantification of signals was performed with ImageJ. Data are expressed as the mean ± SD. ***P < 0.001. Statistical significance was determined by using one-way ANOVA followed by Tukey’s post hoc test. b Immunohistochemical evaluation of F4/80 and CD45 (n = 3 for each experimental group). Scale bar: 50 µm. c Immunoblot analysis of F4/80 (WT, n = 6; mdx, n = 7; mdx+JQ1, n = 7) and CD45 (WT, n = 6; mdx, n = 8; mdx+JQ1, n = 8) in muscles from control, vehicle- and mdx-treated mice. Vinculin serves as a loading control. Lower panel: quantification of signals was performed with ImageJ. Data are expressed as the mean ± SD. **P < 0.01, ***P < 0.001. Statistical significance was determined by using one-way ANOVA followed by Tukey’s post hoc test. d Sirius red staining shows attenuation of fibrosis in JQ1-treated mice (n = 2 sections examined from n = 3 WT animals; mdx mice, n = 5 sections examined from n = 3 mdx-veh and mdx-JQ1 animals). Scale bar: 50 µm. Right panel: quantification of staining. **P < 0.01, ***P < 0.001. Statistical significance was determined by using one-way ANOVA followed by Tukey’s post hoc test. e qRT-PCR analysis of Fibronectin mRNA in TA muscles from control (n = 4), vehicle- (n = 3) and mdx-treated (n = 3) mice. Data are normalized against HPRT and expressed as the mean ± SD. *P < 0.05, ***P < 0.001. Statistical significance was determined by using one-way ANOVA followed by Tukey’s post hoc test. f Immunofluorescence analysis with an antibody raised against the regeneration marker Pax7 in TA cross-sectional section of WT and vehicle- or JQ1-treated mdx mice. n = 3 animals for each experimental group. Scale bar: 25 µm. g Immunoblot analysis of Pax7 (WT, n = 7; mdx, n = 6; mdx+JQ1, n = 7) and eMyHC (WT, n = 3; mdx, n = 3; mdx+JQ1, n = 3) levels in TA of WT, vehicle- and JQ1-treated mdx mice. h Immunoblot analysis of alpha tubulin (WT, n = 4; mdx, n = 5; mdx+JQ1, n = 5), dY tubulin (WT, n = 6; mdx, n = 5; mdx+JQ1, n = 5) and tubulin6 (WT, n = 4; mdx, n = 4; mdx+JQ1, n = 3) levels in TA of WT, vehicle- and JQ1-treated mdx mice. Right panel: quantification of signals was performed with ImageJ. Data represent mean ± SD. *P < 0.05, **P < 0.01, ***P < 0.001. Statistical significance was determined by using one-way ANOVA followed by Tukey’s post hoc test.