The neurobiology of duchenne muscular dystrophy: learning lessons from muscle?

Trends Neurosci. 2000 Mar;23(3):92-9. doi: 10.1016/s0166-2236(99)01510-6.


Several forms of inherited muscular dystrophy are associated with brain abnormalities and cognitive impairment. One of the most common and severe of these diseases is Duchenne muscular dystrophy (DMD). Dystrophin, the product of the DMD gene, is found in neurones, where it is associated with the postsynaptic membrane. Cognitive impairment in individuals with DMD is thought to be due to an abnormality in the neuronal membrane that is caused by lack of dystrophin. Recent experimental evidence has provided valuable clues in our understanding of the complex molecular neurobiology of muscular dystrophy.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • Brain / metabolism
  • Brain / physiopathology*
  • Cognition Disorders / etiology*
  • Cognition Disorders / metabolism
  • Dystrophin / deficiency
  • Dystrophin / genetics
  • Dystrophin / metabolism
  • Humans
  • Mice
  • Multigene Family
  • Muscle, Skeletal / metabolism*
  • Muscle, Skeletal / pathology
  • Muscular Dystrophy, Duchenne / complications*
  • Muscular Dystrophy, Duchenne / genetics
  • Muscular Dystrophy, Duchenne / metabolism
  • Muscular Dystrophy, Duchenne / physiopathology*
  • Neurons / metabolism
  • Nitric Oxide / biosynthesis
  • Retina / physiopathology
  • Synaptic Membranes / metabolism


  • Dystrophin
  • Nitric Oxide