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Fig. 2 | Acta Neuropathologica Communications

Fig. 2

From: rAAV-related therapy fully rescues myonuclear and myofilament function in X-linked myotubular myopathy

Fig. 2

Muscle fibres from XLMTM patients have increased density and irregular spacing of myonuclei, and reduced force-generating capacity. Data from slow/type I muscle fibres from XLMTM patients (MTM1, N = 3) and healthy controls (CTL, N = 4) (n = total of 20 fibres per group). a Representative single muscle fibres from each group, stained for actin (rhodamine phalloidin; red) and nuclei (DAPI; green). The example XLMTM fibre is from the first patient in Table 1. Scale bar: 50 μm. b Proportion of muscle fibres containing internal or central nuclei. Each data point corresponds to the mean per subject. c Number of myonuclei per individual myofibre. d Myonuclear number was linearly related to fibre CSA for all the groups (P < 0.05). e Myonuclear domain volume per individual muscle fibre. f Myonuclear domain size was linearly related to fibre CSA for all the groups (P < 0.05). g Order score (g), an algorithm to assess the regularity of nuclear spacing; a lower score indicates more irregular spacing and more nuclear clustering. h Specific force defined as absolute (maximal) force divided by CSA. Individual data points for c–h correspond to individual muscle fibres. Column graphs show mean ± S.D. Scatter graphs show linear regression lines. Asterisks denote a significant difference compared with CTL (*P < 0.05; **P < 0.01; t-test)

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