Figure 5
From: Myostatin inhibition prevents skeletal muscle pathophysiology in Huntington’s disease mice
ACVR2B/Fc treatment and HTT aggregation. (A) ACVR2B/Fc treatment results in a decrease in the aggregate load in R6/2 TA and quadriceps muscles as assessed by the Seprion ELISA. WT vehicle, n = 5; WT ACVR2B/Fc, n = 10; R6/2 vehicle n = 10; R6/2 ACVR2B/Fc, n = 9. WT background signal derives from the substrate. (B) Number of DAPI stained nuclei per ROI and average nucleus size in DAPI pixels. (C) Number of S830 inclusions per ROI and average inclusion size in pixels. (D) Percentage of S830 signal co-localized with DAPI and percentage of inclusions localized to the nucleus. (E) Number of nuclear S830 inclusions per ROI, the percentage of nuclei with inclusions and average size of nuclear inclusions in pixels (F) Level of expression of Pax7 as fold change from WT. Taqman qPCR values were normalized to the geometric mean of Atp5b, Actb and Sdha. WT vehicle, n = 8; WT ACVR2B/Fc, n = 7; R6/2 vehicle n = 6; R6/2 ACVR2B/Fc, n = 8. Statistical analysis for (B) and (D) was two-way ANOVA with post-hoc Bonferroni correction (see Table S8 for main effects) and for (A), (C), (D) and (E) was Student’s t-test (n = 4/treatment group). Statistical significance for R6/2 vehicle vs R6/2 ACVR2B/Fc is depicted by *p < 0.05; **p < 0.01; ***p < 0.001 and for WT vehicle vs R6/2 vehicle by ###p < 0.001. All data presented ± SEM. WT = wild type, ROI = regions of interest.
