The paradox of muscle hypertrophy in muscular dystrophy

Phys Med Rehabil Clin N Am. 2012 Feb;23(1):149-72, xii. doi: 10.1016/j.pmr.2011.11.014.


Mutations in the dystrophin gene cause Duchenne and Becker muscular dystrophy in humans and syndromes in mice, dogs, and cats. Affected humans and dogs have progressive disease that leads primarily to muscle atrophy. Mdx mice progress through an initial phase of muscle hypertrophy followed by atrophy. Cats have persistent muscle hypertrophy. Hypertrophy in humans has been attributed to deposition of fat and connective tissue (pseudohypertrophy). Increased muscle mass (true hypertrophy) has been documented in animal models. Muscle hypertrophy can exaggerate postural instability and joint contractures. Deleterious consequences of muscle hypertrophy should be considered when developing treatments for muscular dystrophy.

Publication types

  • Review

MeSH terms

  • Animals
  • Contracture / etiology
  • Contracture / physiopathology
  • Disease Models, Animal
  • Dystrophin / genetics
  • Dystrophin / metabolism
  • Humans
  • Hypertrophy / etiology*
  • Hypertrophy / metabolism
  • Hypertrophy / physiopathology
  • Kyphosis / etiology
  • Muscle Strength
  • Muscle, Skeletal / pathology*
  • Muscle, Skeletal / physiopathology
  • Muscular Dystrophies / complications
  • Muscular Dystrophies / drug therapy
  • Muscular Dystrophies / metabolism
  • Muscular Dystrophies / pathology*
  • Muscular Dystrophies / physiopathology
  • Myostatin / antagonists & inhibitors


  • Dystrophin
  • Myostatin