Muscular Dystrophies and the Dystrophin-Glycoprotein Complex

Curr Opin Neurol. 1997 Apr;10(2):168-75. doi: 10.1097/00019052-199704000-00016.

Abstract

Efforts to understand the function of dystrophin, the protein product for the Duchenne muscular dystrophy gene, resulted in the purification of the dystrophin-glycoprotein complex. Over the past year several novel components of this complex have been identified. Recent studies have extended the number of muscular dystrophies associated with the oligomeric complex to six genetically distinct diseases, including three new forms of limb-girdle muscular dystrophy and one form of congenital muscular dystrophy.

Publication types

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

MeSH terms

  • Animals
  • Child
  • Dystrophin / genetics*
  • Dystrophin / physiology
  • Gene Expression / physiology
  • Genotype
  • Humans
  • Membrane Glycoproteins / genetics*
  • Membrane Glycoproteins / physiology
  • Mice
  • Mice, Transgenic
  • Muscular Dystrophies / classification
  • Muscular Dystrophies / genetics*
  • Muscular Dystrophies / physiopathology
  • Phenotype

Substances

  • Dystrophin
  • Membrane Glycoproteins