Muscle function and running activity in mouse models of hereditary muscle dystrophy: impact of double knockout for dystrophin and the transcription factor MyoD

Muscle Nerve. 2012 Apr;45(4):544-51. doi: 10.1002/mus.22318.

Abstract

Introduction: Mice that lack both the transcription factor MyoD and dystrophin display a more pronounced myopathic phenotype when compared with mdx mice. No data on skeletal muscle function and the impact of exercise training are available.

Methods: Six-month-old wild-type, mdx, myoD(-/-), mdx:myoD(+/-), and mdx:myoD(-/-) mice were randomly assigned to either 4 weeks of voluntary running or sedentary behavior. The mdx:myoD(-/-) mice were not able to exercise at all and were kept sedentary.

Results: The soleus muscle of sedentary 7-month-old mdx:myoD(-/-) mice showed a significantly lower force development compared with all other mice. Voluntary running beginning at the age of 6 months led to lower force development of soleus muscle in the mdx animals.

Conclusions: mdx:myoD(-/-) is not a suitable model to study exercise-induced effects on dystrophic muscles. Voluntary exercise in adult mdx mice seems to have detrimental effects on the function of soleus muscle.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Anatomy, Cross-Sectional
  • Animals
  • Disease Progression
  • Dystrophin / genetics*
  • Female
  • Genotype
  • Male
  • Mice
  • Mice, Inbred C57BL
  • Mice, Inbred mdx
  • Mice, Knockout
  • Muscle Contraction / physiology
  • Muscle, Skeletal / pathology
  • Muscle, Skeletal / physiopathology*
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / pathology
  • Muscular Dystrophies / physiopathology*
  • MyoD Protein / genetics*
  • Phenotype
  • Running / physiology*

Substances

  • Dystrophin
  • MyoD Protein
  • MyoD1 myogenic differentiation protein