Figure 3: Muscle pathology from aged mdx and dKO mice.
From: Evidence for ACTN3 as a genetic modifier of Duchenne muscular dystrophy
(a) Hematoxylin and eosin (H&E) stained TA sections from WT, mdx and dKO mice. Scale bar indicates 100 μm. (b) dKO muscles showed a significant reduction in total necrotic area compared with mdx (mdx – 10.7% versus dKO – 4.7%). (c) Both mdx and dKO show an increase in centrally nucleated fibres compared with WT, and dKO show a further increase compared with mdx (dKO – 79% versus mdx 71%, P<0.05). Data shown as mean±s.e.m., One-way ANOVA, *P<0.05, **P<0.01, ***P<0.001. WT n=11, mdx n=12, dKO n=11. (d) Individual fibres were isolated from 12 month old EDLs to assess fibre branching. The left hand panel shows an entire intact fibre from mdx with a branch which originates from the main body of the fibre which re-joins at the mid-belly of the fibre (indicated by arrows). The right hand panels show higher magnification examples of fibre branching. Scale bar indicates 100 μm. (e) Almost all fibres isolated from mdx (99%) and dKO (89%) are branched. (f) However, mdx show a higher number of branches per fibre than dKO; 74% of mdx fibres have 4 or more branches compared with 18% of dKO. Fibre counts are pooled (n=3 mice per genotype) with ∼600 fibres analysed per genotype.
