Figure 9: Physiological improvements of CNM phenotype in severely affected Mtm1 KO mice. | Nature Communications

Figure 9: Physiological improvements of CNM phenotype in severely affected Mtm1 KO mice.

From: Antisense oligonucleotide-mediated Dnm2 knockdown prevents and reverts myotubular myopathy in mice

Figure 9

(a) TA and gast. muscles weight and (b) in situ specific muscle force at week 12 and following 8 weeks of ASO treatment. (c) TA muscle sections were stained with H&E or SDH. Scale bar, 50 μm for histology and 500 nm for TEM. Yellow arrows point to organized triads. (d) Fibre size average was quantified in 400–600 fibres (n=5). (e) TA muscle fibre area distribution of Mtm1KO mice injected with ASO#1 versus WT injected with ASO control (n=5). (f) Fibres with abnormal position of nuclei (n=5). (g) Ratio of triads per sarcomere. (h) DNM2 and GAPDH (loading control) protein levels were measured in TA muscles by western blot. Data represent means±s.e.m. NS, not statistically significant, *P<0.05, **P<0.01, ***P<0.001 (t-test). kDa, kilodalton; MW, molecular weight.

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