Increasing complexity of the dystrophin-associated protein complex

Proc Natl Acad Sci U S A. 1994 Aug 30;91(18):8307-13. doi: 10.1073/pnas.91.18.8307.

Abstract

Duchenne muscular dystrophy is a severe X chromosome-linked, muscle-wasting disease caused by lack of the protein dystrophin. The exact function of dystrophin remains to be determined. However, analysis of its interaction with a large oligomeric protein complex at the sarcolemma and the identification of a structurally related protein, utrophin, is leading to the characterization of candidate genes for other neuromuscular disorders.

Publication types

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

MeSH terms

  • Animals
  • Calcium-Binding Proteins
  • Chromosome Mapping
  • Cytoskeletal Proteins / physiology*
  • Dystroglycans
  • Dystrophin / physiology*
  • Genes
  • Humans
  • Macromolecular Substances
  • Membrane Glycoproteins / physiology*
  • Membrane Proteins / physiology*
  • Muscle Proteins / physiology*
  • Muscles / physiology*
  • Neuromuscular Junction / physiology
  • Utrophin

Substances

  • Calcium-Binding Proteins
  • Cytoskeletal Proteins
  • DAG1 protein, human
  • Dystrophin
  • Macromolecular Substances
  • Membrane Glycoproteins
  • Membrane Proteins
  • Muscle Proteins
  • Utrophin
  • syntrophin alpha1
  • Dystroglycans