Crucial role for the myelin-associated glycoprotein in the maintenance of axon-myelin integrity

Eur J Neurosci. 1995 Mar 1;7(3):511-5. doi: 10.1111/j.1460-9568.1995.tb00347.x.


It has recently been shown that mice deficient in the gene for myelin-associated glycoprotein develop normal myelin sheaths in the peripheral nervous system. Here we report that in mutant mice older than 8 months the maintenance of axon-myelin units is disturbed, resulting in both axon and myelin degeneration. Morphological features include those typically seen in human peripheral neuropathies, where demyelination-induced Schwann cell proliferation and remyelination lead to the formation of so-called onion bulbs. Expression of tenascin-C, a molecule indicative of peripheral nerve degeneration, was up-regulated by axon-deprived Schwann cells and regenerating axons were occasionally seen. Myelin-associated glycoprotein thus appears to play a crucial role in the long-term maintenance of the integrity of both myelin and axons.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Axons / physiology*
  • Brachial Plexus / metabolism
  • Brachial Plexus / ultrastructure
  • Cell Adhesion Molecules, Neuronal / metabolism
  • Extracellular Matrix Proteins / metabolism
  • Femoral Nerve / metabolism
  • Femoral Nerve / ultrastructure
  • Fluorescent Antibody Technique
  • Mice
  • Mice, Knockout
  • Microscopy, Electron
  • Microscopy, Immunoelectron
  • Myelin Proteins / physiology*
  • Myelin Sheath / physiology*
  • Myelin-Associated Glycoprotein
  • Nerve Tissue Proteins / metabolism
  • Sciatic Nerve / metabolism
  • Sciatic Nerve / ultrastructure
  • Tenascin


  • Cell Adhesion Molecules, Neuronal
  • Extracellular Matrix Proteins
  • Myelin Proteins
  • Myelin-Associated Glycoprotein
  • Nerve Tissue Proteins
  • Tenascin