Fig. 6

Delivery of wild type MTM1 gene rescues contractile protein content and force production in XLMTM dogs. Dogs from 5 groups were analysed (N = 3 per group, see legend from Fig. 2). a Mean fluorescence intensity of rhodamine phalloidin (RP) calculated for individual fibres, as a measure of actin density/content. Total myosin (b) and actin (c) protein content in fibres from XLMTM and Healthy dogs, as assessed by proteomics (n = 6 fibres per group). d Myofilament content, as assessed on transmission electron micrographs of longitudinal muscle sections, measured as proportion of the area occupied by myofibrils. Each data point corresponds to the mean per animal. e Representative electron micrographs from each group (Scale bar: 2 μm). f Specific force defined as absolute (maximal) force divided by CSA, and g Rigor force defined as the maximum force divided by CSA in the absence of ATP (15 fibres taken from 3 animals per group). Data points correspond to individual muscle fibres in (a–c, f, g). 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; one-way ANOVA)