Schwann cell myelination occurred without basal lamina formation in laminin alpha2 chain-null mutant (dy3K/dy3K) mice

Glia. 2001 Aug;35(2):101-10. doi: 10.1002/glia.1075.

Abstract

The laminin alpha2 chain is a major component of basal lamina in both skeletal muscle and the peripheral nervous system. Laminin alpha2 chain deficiency causes merosin-deficient congenital muscular dystrophy, which affects not only skeletal muscles, but also the peripheral and central nervous systems. It has been reported that the formation of basal lamina is required for myelination in the peripheral nervous system. In fact, the spinal root of dystrophic mice (dy/dy mice), whose laminin alpha2 chain expression is greatly reduced, shows lack of basal lamina and clusters of naked axons. To investigate the role of laminin alpha2 chain and basal lamina in vivo, we examined the peripheral nervous system of dy3K/dy3K mice, which are null mutants of laminin alpha2 chain. The results indicate the presence of myelination although Schwann cells lacked basal lamina in the spinal roots of dy3K/dy3K mice, suggesting that basal lamina is not an absolute requirement for myelination in vivo. Immunohistochemically, the expression of laminin alpha4 chain was increased and laminin alpha5 chain was preserved in the endoneurium of the spinal root. Laminin alpha4 and alpha5 chains may play the critical role in myelination instead of laminin alpha2 chain in dy3K/dy3K mice. In addition, the motor conduction velocity of the sciatic nerve was significantly reduced compared with that of wild-type littermate. This reduction in conduction velocity may be due to small axon diameter, thin myelin sheath and the patchy disruption of the basal lamina of the nodes of Ranvier in dy3K/dy3K mice.

MeSH terms

  • Animals
  • Axons / metabolism
  • Axons / ultrastructure
  • Basement Membrane / abnormalities*
  • Basement Membrane / growth & development
  • Basement Membrane / ultrastructure
  • Cell Size
  • Immunohistochemistry
  • Laminin / deficiency*
  • Laminin / genetics
  • Laminin / metabolism
  • Lumbar Vertebrae
  • Mice
  • Mice, Knockout
  • Mice, Neurologic Mutants
  • Microscopy, Electron
  • Myelin Sheath / metabolism
  • Myelin Sheath / ultrastructure*
  • Neural Conduction / physiology
  • Peripheral Nerves / abnormalities
  • Peripheral Nerves / growth & development
  • Peripheral Nerves / ultrastructure
  • Schwann Cells / metabolism
  • Schwann Cells / ultrastructure*
  • Sciatic Nerve / abnormalities
  • Sciatic Nerve / growth & development
  • Sciatic Nerve / ultrastructure
  • Spinal Nerve Roots / abnormalities
  • Spinal Nerve Roots / growth & development
  • Spinal Nerve Roots / ultrastructure

Substances

  • Lama4 protein, mouse
  • Laminin
  • laminin alpha 2
  • laminin alpha5