Fig. 3

Engraftment analysis upon cell transplantation in non-injured muscles. a Representative images, capturing the whole engraftment area, show immunostaining for IIH6 (in green) and RFP (in red) in TA muscles from non-injured FKRP^P448L-NSG that had been injected with PBS (upper panel) or mouse ES cell-derived myogenic progenitors (lower panel). DAPI stained nuclei (in blue). Scale bar is 200 μm. b High magnification image of donor-derived engrafted myofibers (from a, white square). Scale bar is 100 μm. c Engraftment quantification based on the number of RFP+/IIH6+ myofibers. Data are shown as mean + SEM from three different cohorts (n = 17; 9 males and 8 females). d Distribution of the number of RFP+/IIH6+ myofibers along the TA muscle (n = 7). e Quantification of the percentage of centrally nucleated myofibers in the PBS injected TA muscles, RFP- and RFP+ area of the cell injected TA muscles. Data are shown as mean + SEM (n = 4; 2 males and 2 females). **p < 0.01. f Representative western blot for IIH6 in TA lysates from 7-week-old FKRP^P448L-NSG mice that have been injected at 3-weeks of age with mouse ES cell-derived myogenic progenitors or PBS (contralateral muscle as negative control) (n = 5; 2 males and 3 females). B6 and NSG muscles were used as reference. β-DG was used as a loading control. g Respective quantification of IIH6 band intensity. e Normalized with β-DG. Data are shown as mean + SEM. **p < 0.01. h Effect of cell transplantation on specific (sF₀: F₀ normalized to CSA) force. Data are shown as mean + SEM (n = 6; 3 males and 3 females). B6 mouse TA muscles were used as a reference (n = 8, 2 males and 6 females). *p < 0.01