Dystrobrevin dynamics in muscle-cell signalling: a possible target for therapeutic intervention in Duchenne muscular dystrophy?

Neuromuscul Disord. 2002 Oct:12 Suppl 1:S110-7. doi: 10.1016/s0960-8966(02)00091-3.

Abstract

The dystrophin-protein complex forms one of the connections between the extracellular matrix and the cytoskeleton of muscle. This link is disrupted in patients with Duchenne and Becker muscular dystrophies. Dystrobrevin is a component of the dystrophin-protein complex that binds to the C-terminus of dystrophin and also to syntrophin. As its name suggests, dystrobrevin is a relative of dystrophin participating in similar intermolecular interactions. Dystrobrevin-deficient mice have a form of muscular dystrophy that leaves the sarcolemma and dystrophin-protein complex intact but affects an as yet unidentified signalling pathway in muscle. Given that the up-regulation of several genes has a beneficial effect on the muscle in some dystrophic mouse models, alpha-dystrobrevin has a number of properties that might be protective in muscular dystrophy. This article discusses the function of dystrobrevin in muscle and reviews its suitability as a therapeutic target for treating patients with Duchenne and Becker muscular dystrophies.

Publication types

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

MeSH terms

  • Animals
  • Cytoskeletal Proteins / metabolism
  • Dystrophin / metabolism
  • Dystrophin-Associated Proteins*
  • Membrane Proteins / metabolism
  • Mice
  • Mice, Inbred mdx
  • Muscle Proteins / metabolism
  • Muscular Dystrophy, Animal / therapy
  • Muscular Dystrophy, Duchenne / metabolism*
  • Muscular Dystrophy, Duchenne / therapy*
  • Neuropeptides / metabolism*
  • Signal Transduction*

Substances

  • Cytoskeletal Proteins
  • Dystrophin
  • Dystrophin-Associated Proteins
  • Membrane Proteins
  • Muscle Proteins
  • Neuropeptides
  • dystrobrevin
  • syntrophin