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Nerve growth factor expression in human dystrophic muscles
Authors:Toti Paolo  Villanova Marcello  Vatti Rosella  Schuerfeld Karin  Stumpo Michela  Barbagli Letizia  Malandrini Alessandro  Costantini Maurizio
Affiliation:Department of Human Pathology and Oncology, University of Siena, Via delle Scotte, 53100 Siena, Italy. paolot@usisi.it
Abstract:Nerve growth factor (NGF) is a neurotrophin that is expressed during muscle development and is also capable of favoring muscle regeneration in experimental studies. The presence of NGF in muscular dystrophies, such as Duchenne and Becker muscular dystrophies, has never been fully explored. By means of immunohistochemistry, we show that regenerating muscle fibers from such patients consistently express NGF, as do myofibroblasts and mast cells. By contrast, rest fibers from dystrophic patients, as well as muscle fibers from healthy, control patients and even regenerative muscle fibers in polymyositis do not show NGF immunoreactivity. The paracrine effect of NGF on muscle regeneration, as well as its chemoattractant capacities for mast cells, may contribute to explaining why regenerating fibers most frequently occur in clusters and why mast cells are more numerous in dystrophic muscles. Moreover, being a mediator of wound healing and tissue fibrosis, NGF may contribute to long-term muscle regeneration impairment by tissue fibrosis in the muscular dystrophies.
Keywords:Becker muscular dystrophy  Duchenne muscular dystrophy  fibrosis  mast cells  regeneration
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