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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