The role of fibrosis in Duchenne muscular dystrophy

Werner Klingler, Karin Jurkat-Rott, Frank Lehmann-Horn, Robert Schleip

Research output: Contribution to journalArticlepeer-review

175 Scopus citations

Abstract

Muscular dystrophies such as Duchenne muscular dystrophy (DMD) are usually approached as dysfunctions of the affected skeletal myofibres and their force transmission. Comparatively little attention has been given to the increase in connective tissue (fibrosis) which accompanies these muscular changes. Interestingly, an increase in endomysial tissue is apparent long before any muscular degeneration can be observed. Fibrosis is the result of a reactive or reparative process involving mechanical, humoral and cellular factors. Originating from vulnerable myofibres, muscle cell necrosis and inflammatory processes are present in DMD. Muscular recovery is limited due to the limited number and capacity of satellite cells. Hence, a proactive and multimodal approach is necessary in order to activate protective mechanisms and to hinder catabolic and tissue degrading pathways. Several avenues are discussed in terms of potential antifibrotic therapy approaches. These include pharmaceutical, nutritional, exercise-based and other mechanostimulatory modalities (such as massage or yoga-like stretching) with the intention of exerting an anti-inflammatory and antifibrotic effect on the affected muscular tissues. A preventive intervention at an early age is crucial, based on the early and seemingly non-reversible nature of the fibrotic tissue changes. Since consistent assessment is essential, different measurement technologies are discussed.

Original languageEnglish
Pages (from-to)184-195
Number of pages12
JournalActa Myologica
Volume31
Issue number3
StatePublished - Dec 2012
Externally publishedYes

Keywords

  • Antifibrotic therapy
  • Duchenne muscular dystrophy
  • Endo- and perimysium
  • Extracellular matrix
  • Fibrosis
  • Myostatin
  • TGF-β1

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