Regular Article

Cell and Tissue Research

, Volume 340, Issue 3, pp 541-548

Elevated satellite cell number in Duchenne muscular dystrophy

  • Michael KottlorsAffiliated withDivision of Neuropediatrics and Muscle Disorders, University Children’s Hospital Freiburg Email author 
  • , Janbernd KirschnerAffiliated withDivision of Neuropediatrics and Muscle Disorders, University Children’s Hospital Freiburg

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Abstract

The regenerative potential of muscle tissue relies mostly on satellite cells situated between the muscular basal membrane and the sarcolemma. The regeneration of muscle tissue comprises proliferation, the propagation of satellite cells, and their subsequent differentiation with the expression of multiple muscle-specific proteins. However, in Duchenne muscular dystrophy (DMD), regeneration cannot compensate for the loss of muscle tissue. To examine the regenerative potential in DMD, satellite cell nuclei number and markers of differentiation in DMD muscle from various disease states were compared with control muscle. Differentiation of satellite cells is characterized by the helix-loop-helix factor myogenin, which is never co-expressed with Pax7, whereas MyoD1 and Myf5 are co-expressed with Pax7, with Myf5 being present even in muscle of controls. The results indicate that satellite cell number is elevated in DMD in comparison with control muscle, even in advanced stages of dystrophy, suggesting that exhaustion of satellite cells is not the primary cause for failed regeneration. The expression of myogenin is correlated neither with fibrosis nor with age. We suggest variable factors influencing the differentiation of satellite cells in DMD.

Keywords

Duchenne muscular dystrophy Satellite cell Regeneration Proliferation Differentiation Human