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Am J Pathol. 2017 Aug;187(8):1814-1827. doi: 10.1016/j.ajpath.2017.04.011. Epub 2017 Jun 12.

Platelet-Derived Growth Factor BB Influences Muscle Regeneration in Duchenne Muscle Dystrophy.

Author information

1
Neuromuscular Disorders Unit, Department of Neurology, Universitat Autònoma de Barcelona, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; Network Research Center in Rare Diseases, Barcelona, Spain.
2
Neuromuscular Disorders Unit, Department of Neurology, Universitat Autònoma de Barcelona, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
3
Network Research Center in Rare Diseases, Barcelona, Spain; Department of Neurology, Hospital Universitario de la Fe, Valencia, Spain.
4
Neuromuscular Disorders Unit, Department of Neurology, Universitat Autònoma de Barcelona, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain; Network Research Center in Rare Diseases, Barcelona, Spain. Electronic address: jdiazm@santpau.cat.

Erratum in

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.

PMID:
28618254
DOI:
10.1016/j.ajpath.2017.04.011
[Indexed for MEDLINE]

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