• Register
Forgot Password

If you have forgotten your password you can enter your email here and get a temporary password sent to your email.


Leaving Community

Are you sure you want to leave this community? Leaving the community will revoke any permissions you have been granted in this community.


Association of dystrophin-related protein with dystrophin-associated proteins in mdx mouse muscle.

Dystrophin is associated with a complex of muscle membrane (sarcolemmal) glycoproteins that provide a linkage to the extracellular matrix protein, laminin. The absence of dystrophin leads to a dramatic reduction of the dystrophin-associated proteins (156DAG, 59DAP, 50DAG, 43DAG and 35DAG) in the sarcolemma of patients with Duchenne muscular dystrophy and mdx mice. Here we demonstrate that dystrophin-related protein (DRP, utrophin), an autosomal homologue of dystrophin, is associated with an identical or antigenically similar complex of sarcolemmal proteins and that DRP and the dystrophin/DRP-associated proteins colocalize to the neuromuscular junction in Duchenne muscular dystrophy and mdx muscle. The DRP and dystrophin/DRP-associated proteins are found throughout the sarcolemma in small-calibre skeletal muscles and cardiac muscle of adult mdx mice. Because these muscles show minimal pathological changes, our results could provide a basis for the upregulation of DRP as a potential therapeutic approach.

Pubmed ID: 1461282


  • Matsumura K
  • Ervasti JM
  • Ohlendieck K
  • Kahl SD
  • Campbell KP



Publication Data

December 10, 1992

Associated Grants


Mesh Terms

  • Animals
  • Centrifugation, Density Gradient
  • Cytoskeletal Proteins
  • Dystrophin
  • Glycoproteins
  • Humans
  • Immunoblotting
  • Immunohistochemistry
  • Membrane Proteins
  • Mice
  • Muscle Proteins
  • Muscles
  • Muscular Dystrophies
  • Muscular Dystrophy, Animal
  • Myocardium
  • Neuromuscular Junction
  • Sarcolemma
  • Tissue Distribution
  • Utrophin