A homologue of dystrophin is expressed at the neuromuscular junctions of normal individuals and DMD patients, and of normal and mdx mice. Immunological evidence

FEBS Lett. 1991 Apr 22;282(1):161-5. doi: 10.1016/0014-5793(91)80468-i.

Abstract

Polyclonal and monoclonal antibodies, which recognize different regions and epitopes of the dystrophin molecule, bind to a protein of Mr 400,000 which is present in extracts of mdx muscle from regions which contain neuromuscular junctions (NMJ) and is absent from those which do not. This NMJ-associated homologue of dystrophin has at least 2 epitopes which are different to usual Xp21 form of dystrophin expressed along the sarcolemma of muscle fibres in normal muscles. This protein is also expressed at the NMJ of a DMD patient who lacks the first 52 exons of the Xp21 dystrophin gene and it must therefore be translated from a different gene transcript.

Publication types

  • Case Reports
  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Antibodies
  • Antibodies, Monoclonal / immunology
  • Blotting, Western
  • Child
  • Dystrophin / biosynthesis*
  • Dystrophin / genetics
  • Epitopes
  • Fluorescent Antibody Technique
  • Humans
  • Male
  • Mice
  • Muscular Dystrophies / metabolism*
  • Neuromuscular Junction / metabolism*

Substances

  • Antibodies
  • Antibodies, Monoclonal
  • Dystrophin
  • Epitopes