title: Satellite cells from dystrophic muscle retain regenerative capacity.
abstract: Duchenne muscular dystrophy is an inherited disorder that is characterized by progressive skeletal muscle weakness and wasting, with a failure of muscle maintenance/repair mediated by satellite cells (muscle stem cells). The function of skeletal muscle stem cells resident in dystrophic muscle may be perturbed by being in an increasing pathogenic environment, coupled with constant demands for repairing muscle. To investigate the contribution of satellite cell exhaustion to this process, we tested the functionality of satellite cells isolated from the mdx mouse model of Duchenne muscular dystrophy. We found that satellite cells derived from young mdx mice contributed efficiently to muscle regeneration within our in vivo mouse model. To then test the effects of long-term residence in a dystrophic environment, satellite cells were isolated from aged mdx muscle. Surprisingly, they were as functional as those derived from young or aged wild type donors. Removing satellite cells from a dystrophic milieu reveals that their regenerative capacity remains both intact and similar to satellite cells derived from healthy muscle, indicating that the host environment is critical for controlling satellite cell function.
authors.affiliation: University College London, Institute of Child Health, The Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neurosciences Programme, 30 Guilford Street, London WC1N 1EH, United Kingdom. Electronic address: l.boldrin@ucl.ac.uk.
authors.forename: Luisa
authors.initials: L
authors.lastname: Boldrin
authors.affiliation: King's College London, The Randall Division of Cell and Molecular Biophysics, New Hunt's House, London SE1 1UL, United Kingdom.
authors.forename: Peter S
authors.initials: PS
authors.lastname: Zammit
authors.affiliation: University College London, Institute of Child Health, The Dubowitz Neuromuscular Centre, Molecular Neurosciences Section, Developmental Neurosciences Programme, 30 Guilford Street, London WC1N 1EH, United Kingdom. Electronic address: jennifer.morgan@ucl.ac.uk.
authors.forename: Jennifer E
authors.initials: JE
authors.lastname: Morgan
mesh_terms: D000375:Aging; D000818:Animals; D002478:Cells, Cultured; D004195:Disease Models, Animal; D051379:Mice; D008810:Mice, Inbred C57BL; D018101:Mice, Inbred mdx; D008819:Mice, Nude; D009132:Muscles / Q000473:pathology / Q000502:physiology*; D020388:Muscular Dystrophy, Duchenne / Q000378:metabolism / Q000473:pathology / Q000628:therapy; D012038:Regeneration; D032496:Satellite Cells, Skeletal Muscle / Q000166:cytology / Q000378:metabolism / Q000637:transplantation*