Skeletal muscles fibers are formed by the fusion of hundreds of cells where myonuclei are actively spread at myofibers periphery. Growing evidences supports connections between myonuclear positioning and muscle functionality. How myonuclei localization in muscle fibers contributes to muscle efficiency remains poorly characterized. Here, we show that in developing myofibers, SH3KBP1 scaffolds Endoplasmic Reticulum through […]

Skeletal muscle fibers are large syncytia but it is currently unknown whether gene expression is coordinately regulated in their numerous nuclei. By snRNA-seq and snATAC-seq, we showed that slow, fast, myotendinous and neuromuscular junction myonuclei each have different transcriptional programs, associated with distinct chromatin states and combinations of transcription factors. In adult mice, identified myofiber […]

Duchenne muscular dystrophy (DMD) is a devastating neuromuscular disease caused by an absence of the dystrophin protein, which is essential for muscle fiber integrity. Among the developed therapeutic strategies for DMD, the exon-skipping approach corrects the frameshift and partially restores dystrophin expression. It could be achieved through the use of antisense sequences, such as peptide-conjugated […]