Lack of dystrophin disrupts fetal muscle stem cell polarity and commitment during secondary myogenesis
No cure exists for Duchenne Muscular Dystrophy (DMD), a devastating and fatal muscle degenerative disease caused by a loss-of-function mutations to the DMD gene which encode the large cytoskeleton protein dystrophin. Conventionally, the disease is thought to manifest around 3-5 years of age when fragile myofibers become damaged from patient ambulation. However, we find that perturbation in fetal muscle stem cell (fMuSC) commitment and polarity occur during secondary myogenesis in the mdx mouse, a model of DMD. To study the cell state and the transcriptomic profileof mdx fMuSCs, we conducted single cell RNA-sequencing of myogenic cells derived from the limbs of E17.5 Pax7-nGFP embryos on the C57BL/10ScSnJ and mdx backgrounds. Overall design: Fetal muscle stem cells were FACS-isolated from the limbs of the limbs of E17.5 Pax7-nGFP embryos on the C57BL/10ScSnJ and mdx backgrounds.The experiment was performed on biological duplicates.