Peripheral demyelination and neuropathic pain behavior in periaxin-deficient mice

Neuron. 2000 May;26(2):523-31. doi: 10.1016/s0896-6273(00)81184-8.


The Prx gene in Schwann cells encodes L- and S-periaxin, two abundant PDZ domain proteins thought to have a role in the stabilization of myelin in the peripheral nervous system (PNS). Mice lacking a functional Prx gene assemble compact PNS myelin. However, the sheath is unstable, leading to demyelination and reflex behaviors that are associated with the painful conditions caused by peripheral nerve damage. Older Prx-/- animals display extensive peripheral demyelination and a severe clinical phenotype with mechanical allodynia and thermal hyperalgesia, which can be reversed by intrathecal administration of a selective NMDA receptor antagonist We conclude that the periaxins play an essential role in stabilizing the Schwann cell-axon unit and that the periaxin-deficient mouse will be an important model for studying neuropathic pain in late onset demyelinating disease.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Axons / ultrastructure
  • Behavior, Animal*
  • Demyelinating Diseases / complications*
  • Demyelinating Diseases / genetics
  • Demyelinating Diseases / pathology
  • Electrophysiology
  • Excitatory Amino Acid Antagonists / pharmacology
  • Humans
  • Hyperalgesia / genetics
  • Hyperalgesia / physiopathology
  • Membrane Proteins / deficiency*
  • Membrane Proteins / genetics
  • Mental Disorders / etiology
  • Mice
  • Mice, Knockout / genetics
  • Neural Conduction
  • Pain / etiology*
  • Pain / psychology*
  • Peripheral Nerves / physiopathology
  • Peripheral Nervous System Diseases / complications*
  • Peripheral Nervous System Diseases / genetics
  • Peripheral Nervous System Diseases / pathology
  • Schwann Cells / ultrastructure
  • Somatosensory Disorders / genetics
  • Somatosensory Disorders / physiopathology


  • Excitatory Amino Acid Antagonists
  • Membrane Proteins
  • periaxin