title: The increase of pericyte population in human neuromuscular disorders supports their role in muscle regeneration in vivo.
abstract: Pericytes are periendothelial cells that have been involved in many different functions including a possible role as mesodermal stem/progenitor cells. In the present study we demonstrate that alkaline phosphatase (AP) expression is specific for human muscular pericytes and can be used as a marker to identify them in skeletal muscle biopsies. We studied the pericyte population in skeletal muscle biopsies from controls, myopathic and neuropathic patients. We observed a significant increase in the number of pericytes only in myopathies that correlated with the number of NCAM(+) fibres, suggesting that an active muscular degenerative/regenerative process is related to an increase in the pericyte population. AP(+) pericytes sorted from skeletal muscle samples were able to activate the myogenic programme and fuse with both mononucleate satellite cells and mature multinucleated myotubes in vitro, demonstrating that they could participate in muscle regeneration. In accordance, pericytes expressing the myogenic transcription factor MyoD were found in biopsies of myopathic biopsies. All these data support the hypothesis that, apart from satellite cells, pericytes may play an important role in muscle regeneration in adult human muscles in vivo.
authors: Neuromuscular Disorders Unit, Neurology Department, Universitat Autònoma de Barcelona, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; Centro de Investigación Biomédica en Red en Enfermedades Neurodegenerativas (CIBERNED), Madrid, Spain.
authors: Jordi
authors: J
authors: Díaz-Manera
authors: Eduard
authors: E
authors: Gallardo
authors: Noemi
authors: N
authors: de Luna
authors: Miquel
authors: M
authors: Navas
authors: Laura
authors: L
authors: Soria
authors: Matteo
authors: M
authors: Garibaldi
authors: Ricard
authors: R
authors: Rojas-García
authors: Rossana
authors: R
authors: Tonlorenzi
authors: Giulio
authors: G
authors: Cossu
authors: Isabel
authors: I
authors: Illa
mesh_terms: D000328:Adult; D015415:Biomarkers; D001706:Biopsy; D002454:Cell Differentiation / Q000502:physiology; D002478:Cells, Cultured; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018485:Muscle Fibers, Skeletal / Q000166:cytology / Q000502:physiology; D018482:Muscle, Skeletal / Q000166:cytology* / Q000502:physiology*; D009136:Muscular Dystrophies / Q000473:pathology* / Q000503:physiopathology; D017570:MyoD Protein / Q000378:metabolism; D009468:Neuromuscular Diseases / Q000473:pathology* / Q000503:physiopathology; D020286:Pericytes / Q000166:cytology*; D012038:Regeneration / Q000502:physiology*; D032496:Satellite Cells, Skeletal Muscle / Q000166:cytology / Q000502:physiology
chemical_list: D015415:Biomarkers; D017570:MyoD Protein; C057363:MyoD1 myogenic differentiation protein