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A new role for dystrophin in muscle stem cells

Nature Medicine volume 21, pages 1391–1393 (2015)Cite this article

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Duchenne muscular dystrophy (DMD) is a devastating X-linked disease that is characterized by progressive muscle degeneration and caused by mutations in dystrophin. Dystrophin is critical for myofiber structural integrity, but a new study reveals an additional important role for this protein in muscle stem cells.

DMD was first described in the 1860s by the French neurologist Guillaume Duchenne as a disease of progressive muscle weakness in which muscle fibers are frail and surrounded by abundant fibrotic connective tissue1. Mutations in the gene encoding dystrophin, DMD, are responsible for causing DMD. Dystrophin is a rod-shaped protein that is expressed primarily by differentiated myofibers2. Dystrophin, in combination with a complex of other proteins, connects the intracellular cytoskeleton of a myofiber through the cell membrane to the surrounding extracellular matrix; it thus stabilizes the myofiber membrane during muscle contraction. Mutations in DMD lead to muscle cell membrane fragility, contraction-induced damage and muscle degeneration. Chronic, successive rounds of muscle degeneration and regeneration with attendant fibrosis and inflammation lead to a loss of muscle mass and function and, ultimately, death. To date, work on the role of dystrophin in muscles has focused exclusively on its requirement in differentiated muscle fibers. However, Dumont et al.3 now show a surprising new role for dystrophin—one in muscle stem cells.

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Figure 1: The role of dystrophin in satellite cells in Duchenne muscular dystrophy (DMD).

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  1. Alexandra C. Keefe and Gabrielle Kardon are in the Department of Human Genetics, University of Utah, Salt Lake City, Utah, USA.,

    Alexandra C Keefe & Gabrielle Kardon

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  1. Alexandra C Keefe
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  2. Gabrielle Kardon
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Correspondence to Gabrielle Kardon.

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Keefe, A., Kardon, G. A new role for dystrophin in muscle stem cells. Nat Med 21, 1391–1393 (2015). https://doi.org/10.1038/nm.4006

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