Duchenne muscular dystrophy (DMD) is characterized by a progressive Loss of muscle fibers, and their substitution by fibrotic and adipose tissue. Many factors contribute to this process, but the molecular pathways related to regeneration and degeneration of muscle are not completely known. Platelet derived growth factor (PDGF)-BB belongs to a family of growth factors that regulate proliferation, migration, and differentiation of mesenchymal cells. The role of PDGF-BB in muscle regeneration in humans has not been studied. We analyzed the expression of PDGF-BB in muscle biopsy samples from controls and patients with DMD. We performed in vitro experiments to understand the effects of PDGF-BB on myoblasts involved in the pathophysiology of muscular dystrophies and confirmed our results in vivo by treating the mdx murine model of DMD with repeated i.m. injections of PDGF-BB. We observed that regenerating and necrotic muscle fibers in muscle biopsy samples from DMD patients expressed PDGF-BB. In vitro, PDGF-BB attracted myoblasts and activated their proliferation. Analysis of muscles from the animals treated with PDGF-BB showed an increased population of satellite cells and an increase in the number of regenerative fibers, with a reduction in inflammatory infiltrates, compared with those in vehicle-treated mice. Based on our results, PDGF-BB may play a protective role in muscular dystrophies by enhancing muscle regeneration through activation of satellite cell proliferation and migration.

Platelet-derived growth factor BB influences muscle regeneration in Duchenne muscle dystrophy / Pinol-Jurado, P.; Gallardo, E.; de Luna, N.; Suarez-Calvet, X.; Sanchez-Riera, C.; Fernandez-Simon, E.; Gomis, C.; Illa, I.; Diaz-Manera, J.. - In: THE AMERICAN JOURNAL OF PATHOLOGY. - ISSN 0002-9440. - 187:8(2017), pp. 1814-1827. [10.1016/j.ajpath.2017.04.011]

Platelet-derived growth factor BB influences muscle regeneration in Duchenne muscle dystrophy

Sanchez-Riera C.
Membro del Collaboration Group
;
2017

Abstract

Duchenne muscular dystrophy (DMD) is characterized by a progressive Loss of muscle fibers, and their substitution by fibrotic and adipose tissue. Many factors contribute to this process, but the molecular pathways related to regeneration and degeneration of muscle are not completely known. Platelet derived growth factor (PDGF)-BB belongs to a family of growth factors that regulate proliferation, migration, and differentiation of mesenchymal cells. The role of PDGF-BB in muscle regeneration in humans has not been studied. We analyzed the expression of PDGF-BB in muscle biopsy samples from controls and patients with DMD. We performed in vitro experiments to understand the effects of PDGF-BB on myoblasts involved in the pathophysiology of muscular dystrophies and confirmed our results in vivo by treating the mdx murine model of DMD with repeated i.m. injections of PDGF-BB. We observed that regenerating and necrotic muscle fibers in muscle biopsy samples from DMD patients expressed PDGF-BB. In vitro, PDGF-BB attracted myoblasts and activated their proliferation. Analysis of muscles from the animals treated with PDGF-BB showed an increased population of satellite cells and an increase in the number of regenerative fibers, with a reduction in inflammatory infiltrates, compared with those in vehicle-treated mice. Based on our results, PDGF-BB may play a protective role in muscular dystrophies by enhancing muscle regeneration through activation of satellite cell proliferation and migration.
2017
duchenne; muscle regeneration; muscle dystrophy; PDGFRb
01 Pubblicazione su rivista::01a Articolo in rivista
Platelet-derived growth factor BB influences muscle regeneration in Duchenne muscle dystrophy / Pinol-Jurado, P.; Gallardo, E.; de Luna, N.; Suarez-Calvet, X.; Sanchez-Riera, C.; Fernandez-Simon, E.; Gomis, C.; Illa, I.; Diaz-Manera, J.. - In: THE AMERICAN JOURNAL OF PATHOLOGY. - ISSN 0002-9440. - 187:8(2017), pp. 1814-1827. [10.1016/j.ajpath.2017.04.011]
File allegati a questo prodotto
File Dimensione Formato  
Piñol-Jurado_Platelet-derived_2017.pdf

solo gestori archivio

Tipologia: Versione editoriale (versione pubblicata con il layout dell'editore)
Licenza: Tutti i diritti riservati (All rights reserved)
Dimensione 4.91 MB
Formato Adobe PDF
4.91 MB Adobe PDF   Contatta l'autore

I documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11573/1678491
Citazioni
  • ???jsp.display-item.citation.pmc??? 17
  • Scopus 34
  • ???jsp.display-item.citation.isi??? 32
social impact