title: Innervation of dystrophic muscle after muscle stem cell therapy.
abstract: Duchenne muscular dystrophy (DMD) is caused by loss of the structural protein, dystrophin, resulting in muscle fragility. Muscle stem cell (MuSC) transplantation is a potential therapy for DMD. It is unknown whether donor-derived muscle fibers are structurally innervated.
  
  
  
  Green fluorescent protein (GFP)-expressing MuSCs were transplanted into the tibials anterior of adult dystrophic mdx/mTR mice. Three weeks later the neuromuscular junction was labeled by immunohistochemistry.
  
  
  
  The percent overlap between pre- and postsynaptic immunolabeling was greater in donor-derived GFP(+) myofibers, and fewer GFP(+) myofibers were identified as denervated compared with control GFP(-) fibers (P = 0.001 and 0.03). GFP(+) fibers also demonstrated acetylcholine receptor fragmentation and expanded endplate area, indicators of muscle reinnervation (P = 0.008 and 0.033).
  
  
  
  It is unclear whether GFP(+) fibers are a result of de novo synthesis or fusion with damaged endogenous fibers. Either way, donor-derived fibers demonstrate clear histological innervation. Muscle Nerve 54: 763-768, 2016.
authors.affiliation: Development, Aging and Regeneration Program, Sanford Children's Health Research Center, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, California, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Tierney
authors.affiliation: Department of Rehabilitation Science, School of Public Health and Health Professions, Kimball Tower, 3435 Main Street, Buffalo, New York, 14214-3079, USA.
authors.forename: Christina
authors.initials: C
authors.lastname: Garcia
authors.affiliation: Department of Rehabilitation Science, School of Public Health and Health Professions, Kimball Tower, 3435 Main Street, Buffalo, New York, 14214-3079, USA.
authors.forename: Matthew
authors.initials: M
authors.lastname: Bancone
authors.affiliation: Development, Aging and Regeneration Program, Sanford Children's Health Research Center, Sanford Burnham Prebys Medical Discovery Institute, La Jolla, California, USA.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Sacco
authors.affiliation: Department of Rehabilitation Science, School of Public Health and Health Professions, Kimball Tower, 3435 Main Street, Buffalo, New York, 14214-3079, USA. kep7@buffalo.edu.|Program in Neuroscience, School of Medicine and Biomedical Sciences, University at Buffalo, Buffalo, New York, USA. kep7@buffalo.edu.
authors.forename: Kirkwood E
authors.initials: KE
authors.lastname: Personius
mesh_terms: D000818:Animals; D051379:Mice; D018101:Mice, Inbred mdx; D008822:Mice, Transgenic; D032342:Muscle Cells / Q000737:chemistry / Q000637:transplantation*; D018485:Muscle Fibers, Skeletal / Q000737:chemistry / Q000637:transplantation; D018482:Muscle, Skeletal / Q000737:chemistry / Q000294:innervation*; D009137:Muscular Dystrophy, Animal / Q000503:physiopathology / Q000628:therapy; D020388:Muscular Dystrophy, Duchenne / Q000503:physiopathology* / Q000628:therapy*; D033581:Stem Cell Transplantation / Q000379:methods*
keywords: Duchenne muscular dystrophy; denervation; muscle stem cell; neuromuscular junction; skeletal muscle