Adrien Morin, Amalia Stantzou, Olga N Petrova, John Hildyard, Thomas Tensorer, Meriem Matouk, Mina V Petkova, Isabelle Richard, Tudor Manoliu, Aurélie Goyenvalle, Sestina Falcone, Markus Schuelke, Corinne Laplace-Builhé, Richard J Piercy, Luis Garcia, Helge Amthor
Dystrophin is essential for muscle health: its sarcolemmal absence causes the fatal, X-linked condition, Duchenne muscular dystrophy (DMD). However, its normal, spatial organization remains poorly understood, which hinders the interpretation of efficacy of its therapeutic restoration. Using female reporter mice heterozygous for fluorescently tagged dystrophin ( DmdEGFP ), we here reveal that dystrophin distribution is unexpectedly compartmentalized, being restricted to myonuclear-defined sarcolemmal territories extending ~80 µm, which we called "basal sarcolemmal dystrophin units (BSDUs)...
January 10, 2023: Proceedings of the National Academy of Sciences of the United States of America