Differential heparin sensitivity of alpha-dystroglycan binding to laminins expressed in normal and dy/dy mouse skeletal muscle

J Biol Chem. 1998 Sep 11;273(37):24139-44. doi: 10.1074/jbc.273.37.24139.

Abstract

The alpha-dystroglycan binding properties of laminins extracted from fully differentiated skeletal muscle were characterized. We observed that the laminins expressed predominantly in normal adult rat or mouse skeletal muscle bound alpha-dystroglycan in a Ca2+-dependent, ionic strength-sensitive, but heparin-insensitive manner as we had observed previously with purified placental merosin (Pall, E. A., Bolton, K. M., and Ervasti, J. M. 1996 J. Biol. Chem. 271, 3817-3821). Rat skeletal muscle laminins partially purified by heparin-agarose affinity chromatography also bound alpha-dystroglycan without sensitivity to heparin. We also confirm previous studies of dystrophic dy/dy mouse skeletal muscle showing that the alpha2 chain of merosin is reduced markedly and that the laminin alpha1 chain is not up-regulated detectably. However, we further observed a quantitative decrease in the expression of laminin beta/gamma chain immunoreactivity in alpha2 chain-deficient dy/dy skeletal muscle and reduced alpha-dystroglycan binding activity in laminin extracts from dy/dy muscle. Most interestingly, the alpha-dystroglycan binding activity of residual laminins expressed in merosin-deficient dy/dy skeletal muscle was inhibited dramatically (69 +/- 19%) by heparin. These results identify a potentially important biochemical difference between the laminins expressed in normal and dy/dy skeletal muscle which may provide a molecular basis for the inability of other laminin variants to compensate fully for the deficiency of merosin in some forms of muscular dystrophy.

Publication types

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

MeSH terms

  • Animals
  • Chromatography, Affinity
  • Cytoskeletal Proteins / isolation & purification
  • Cytoskeletal Proteins / metabolism*
  • Dystroglycans
  • Female
  • Gene Expression Regulation
  • Heparin / metabolism
  • Heparin / pharmacology*
  • Laminin / genetics
  • Laminin / isolation & purification
  • Laminin / metabolism*
  • Membrane Glycoproteins / isolation & purification
  • Membrane Glycoproteins / metabolism*
  • Mice
  • Mice, Neurologic Mutants
  • Muscle, Skeletal / metabolism*
  • Muscular Dystrophy, Animal / genetics
  • Muscular Dystrophy, Animal / metabolism*
  • Protein Binding / drug effects
  • Rabbits
  • Rats
  • Rats, Sprague-Dawley
  • Receptors, Laminin / metabolism
  • Reference Values

Substances

  • Cytoskeletal Proteins
  • Laminin
  • Membrane Glycoproteins
  • Receptors, Laminin
  • Dystroglycans
  • Heparin