Ultrastructural evidence of neurofilament involvement in immune-mediated motor neuron injury

Neurol Res. 2008 Nov;30(9):990-4. doi: 10.1179/016164108X323780. Epub 2008 Jul 25.

Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive neurological disorder. A pathologic hallmark of ALS is accumulation of neurofilaments in proximal axons of affected motor neurones. As the neurofilaments involved in immune-mediated spinal cord ventral horn motor neuron degeneration and loss, we developed immune-mediated motor neuron injury animal model by inoculating Lewis rats with swine spinal cord homogenate and investigated the ultrastructural features of neurofilament accumulation using transmission electron microscopy. Our results showed that there was aberrant accumulation of neurofilaments in perikarya and processes of remaining motor neurons in recipient animals, which is similar to those observed in ALS patients. These findings suggest that immune-mediated motor neuron injury may share a common pathogenesis with ALS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / immunology
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • Animals
  • Disease Models, Animal
  • Male
  • Microscopy, Electron, Transmission
  • Motor Neuron Disease / immunology
  • Motor Neuron Disease / metabolism*
  • Motor Neuron Disease / pathology
  • Motor Neurons / metabolism*
  • Motor Neurons / pathology
  • Motor Neurons / ultrastructure
  • Neurofilament Proteins / metabolism*
  • Neurofilament Proteins / ultrastructure
  • Rats
  • Rats, Inbred Lew
  • Spinal Cord / metabolism
  • Spinal Cord / pathology
  • Spinal Cord / ultrastructure
  • Swine
  • Tissue Extracts / metabolism

Substances

  • Neurofilament Proteins
  • Tissue Extracts