Fig. 2

Validation of FKRP^P448L-NSG mice as a model for transplantation studies. a Representative images show immunostaining for IIH6 (in green) and RFP (in red) in muscle sections from FKRP^P448L (upper panel) and FKRP^P448L-NSG (lower panel) mice that had been pre-injured with CTX and injected with mouse ES cell-derived myogenic progenitors in the right leg and PBS in the contralateral leg. DAPI stained nuclei (in blue). Scale bar is 50 μm. b Engraftment quantification based on the number of RFP+/IIH6+ myofibers (from a). Data are shown as mean + SEM (n = 4; 4 females for FKRP^P448L, and 2 males and 2 females for FKRP^P448L-NSG). c Representative images show immunostaining for IIH6 (in green) and human LAMIN A/C (in red) in muscle sections from FKRP^P448L-NSG that had been injected with human iPS cell-derived myogenic progenitors (lower panel) or PBS (upper panel). DAPI stained nuclei (in blue). Scale bar is 50 μm. d Engraftment quantification based on the number of IIH6+/LAMIN A/C+ myofibers (from c). Data are shown as mean + SEM from 2 independent cohorts (n = 8; 6 males and 2 females)