Fig. 1

∆Ex50-Dmd-Luc mouse model. a Strategy for creation of dystrophin reporter mice. Dystrophin (Dmd) gene with exons is indicated in blue. Using CRISPR/Cas9 mutagenesis, we inserted a DNA cassette encoding the luciferase reporter with the protease 2A cleavage site at the 3′ end of the dystrophin coding region. b Bioluminescence imaging of wild-type (WT) and Dmd knock-in luciferase reporter (referred as WT-Dmd-Luc) mice. c Strategy for creation of ΔEx50-Dmd-Luc reporter mice. Dystrophin (Dmd) gene with exons is indicated in blue. Using CRISPR/Cas9 mutagenesis, we deleted the exon 50 of Dmd gene. d Western blot analysis of dystrophin (DMD), luciferase and vinculin (VCL) expression in skeletal muscle and heart tissues. e Bioluminescence imaging of wild-type (WT), WT-Dmd-Luc and ΔEx50-Dmd-Luc reporter mice