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

Nature. 1992 Dec 10;360(6404):588-91. doi: 10.1038/360588a0.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

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


  • Cytoskeletal Proteins
  • Dystrophin
  • Glycoproteins
  • Membrane Proteins
  • Muscle Proteins
  • Utrn protein, mouse
  • Utrophin