title: Satellite Cells in Muscular Dystrophy - Lost in Polarity.
abstract: Recent findings employing the mdx mouse model for Duchenne muscular dystrophy (DMD) have revealed that muscle satellite stem cells play a direct role in contributing to disease etiology and progression of DMD, the most common and severe form of muscular dystrophy. Lack of dystrophin expression in DMD has critical consequences in satellite cells including an inability to establish cell polarity, abrogation of asymmetric satellite stem-cell divisions, and failure to enter the myogenic program. Thus, muscle wasting in dystrophic mice is not only caused by myofiber fragility but is exacerbated by intrinsic satellite cell dysfunction leading to impaired regeneration. Despite intense research and clinical efforts, there is still no effective cure for DMD. In this review we highlight recent research advances in DMD and discuss the current state of treatment and, importantly, how we can incorporate satellite cell-targeted therapeutic strategies to correct satellite cell dysfunction in DMD.
authors.affiliation: Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada.
authors.forename: Natasha C
authors.initials: NC
authors.lastname: Chang
authors.affiliation: Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada.
authors.forename: Fabien P
authors.initials: FP
authors.lastname: Chevalier
authors.affiliation: Sprott Centre for Stem Cell Research, Regenerative Medicine Program, Ottawa Hospital Research Institute, Ottawa, ON, K1H 8L6, Canada; Department of Cellular and Molecular Medicine, Faculty of Medicine, University of Ottawa, Ottawa, ON, K1H 8M5, Canada. Electronic address: mrudnicki@ohri.ca.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Rudnicki
mesh_terms: D000818:Animals; D016764:Cell Polarity; D049109:Cell Proliferation; D016189:Dystrophin / Q000235:genetics / Q000378:metabolism; D015316:Genetic Therapy; D006801:Humans; D051379:Mice; D018101:Mice, Inbred mdx; D024510:Muscle Development; D018482:Muscle, Skeletal / Q000254:growth & development* / Q000378:metabolism*; D009137:Muscular Dystrophy, Animal; D020388:Muscular Dystrophy, Duchenne / Q000473:pathology* / Q000503:physiopathology* / Q000628:therapy; D032496:Satellite Cells, Skeletal Muscle / Q000502:physiology*
chemical_list: D016189:Dystrophin
keywords: Duchenne muscular dystrophy; Mark2; Par1b; Pard3; asymmetric division; dystrophin; regenerative myogenesis; satellite cells; stem cell polarity