Figure 5: Physiological effects of ASO-Dnm2 systemic injections at 12 weeks of age.
From: Antisense oligonucleotide-mediated Dnm2 knockdown prevents and reverts myotubular myopathy in mice
(a) TA and gast. muscle weights from these mice were measured and the ratio relative to the body weight was represented. (b) The TA in situ muscle force was measured after sciatic nerve stimulation. The specific muscle force was calculated by dividing the absolute force by the TA weight. (c) TA muscle transversal sections stained with H&E (left panel) and SDH (middle panel) at 12 weeks (10 weeks of treatment). Sarcomere and triads (yellow arrows) ultrastructure assessed by TEM (right panel). Scale bars for H&E and SDH is 50 μm and 500 nm for TEM pictures. (d) The percentage of fibres with mislocalized nuclei was counted on 1,000 fibres per group. (e) TA muscle fibre area was calculated on 300–600 fibres per sample. For all tests cited above, n=5–7 mice per group except for Mtm1KO treated with 3.125 mg kg−1 where n=3. Data represent means±s.e.m. NS, no statistical significance, *P<0.05, **P<0.01, ***P<0.001 for mice treated with ASO#1 versus WT treated with ASO control (ctrl) (ANOVA test followed by post hoc Bonferroni).
