Increase in decorin and biglycan in Duchenne muscular dystrophy: Role of fibroblasts as cell source of these proteoglycans in the disease

Ricardo Fadic, Valeria Mezzano, Karin Alvarez, Daniel Cabrera, Jenny Holmgren, Enrique Brandan*

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

78 Scopus citations

Abstract

Fibrosis is a common pathological feature observed in muscles of patients with Duchenne muscular dystrophy (DMD). Biglycan and decorin are small chondroitin/dermatan sulfate proteoglycans in the muscle extracellular matrix (ECM) that belong to the family of structurally related proteoglycans called small leucine-rich repeat proteins. Decorin is considered an anti-fibrotic agent, preventing the process by blocking TGF-β activity. There is no information about their expression in DMD patients. We found an increased amount of both proteoglycans in the ECM of skeletal muscle biopsies obtained from DMD patients. Both biglycan and decorin were augmented in the perimysium of muscle tissue, but only decorin increased in the endomysium as seen by immunohistochemical analyses. Fibroblasts were isolated from expiants obtained from muscle of DMD patients and the incorporation of radioactive sulfate showed an increased synthesis of both decorin and biglycan in cultured fibroblasts compared to controls. The size of decorin and biglycan synthesized by DMD and control fibroblasts seems to be similar in size and anion charge. These findings show that decorin and biglycan are increased in DMD skeletal muscle and suggest that fibroblasts would be, at least, one source for these proteoglycans likely playing a role in the muscle response to dystrophic cell damage.

Original languageEnglish
Pages (from-to)758-769
Number of pages12
JournalJournal of Cellular and Molecular Medicine
Volume10
Issue number3
DOIs
StatePublished - 2006
Externally publishedYes

Keywords

  • Biglycan
  • Decorin
  • Duchenne muscular dystrophy
  • Interstitial fibrosis
  • Proteoglycans

Fingerprint

Dive into the research topics of 'Increase in decorin and biglycan in Duchenne muscular dystrophy: Role of fibroblasts as cell source of these proteoglycans in the disease'. Together they form a unique fingerprint.

Cite this