(A) Schematic representation of the experimental design. (B) Representative images of a low- and a high-grade chimera, as well as a complemented chimera. Black coat color indicates iPSC chimeric contribution in low- and high-grade chimeras. Scale bars: 3 cm. (C) A graph showing chimera numbers based on coat color or Pax7-nGFP allele genotyping. (D) Representative FACS plots displaying the percentage of Pax7-nGFP⁺ cells within the ITGA7⁺ satellite cell population of the indicated animals. (E) A graph showing the quantification of the FACS plot shown in D for a larger group of analyzed mice. n = 3 animals for non-complemented chimeras and n = 5 animals for complemented chimeras as well as Pax7-nGFP control mice. Data are presented as mean ± SD. Statistical analysis was performed using an ordinary 1-way ANOVA with Tukey’s multiple-comparison test. ****P ≤ 0.0001. (F) PCR for dystrophin using DNA extracted from the specified cell populations and animals. (G) Schematic representation of the isolation and expansion of myoblasts from the muscles of complemented chimeras. (H) PCR for dystrophin in total muscles of the specified mice prior to satellite cell isolation. (I) Representative FACS plots showing Pax7-nGFP expression in muscles of the indicated animals. (J) A graph showing quantification of the analysis shown in I. n = 5 animals for each group. Data are presented as mean ± SD. Statistical analysis was performed using a Student’s 2-tailed t test. (K) Representative images of chimera-derived edited Dmd^mdx; Pax7-nGFP⁺ myoblasts. Scale bars: 100 μm. (L) PCR for dystrophin using DNA extracted from FACS-purified Dmd^mdx; Pax7-nGFP⁺ myoblasts. Note the presence of only an edited band. (M) Immunostaining for the indicated markers in myoblast-derived myotubes from the specified cell lines. Scale bars: 100 μm.