An astrocyte regenerative response from vimentin-containing cells in the spinal cord of amyotrophic lateral sclerosis's disease-like transgenic (G93A SOD1) mice

Neurodegener Dis. 2015;15(1):1-12. doi: 10.1159/000369466. Epub 2015 Jan 15.

Abstract

The reason for regeneration in the adult spinal cord during motor neuron degeneration in amyotrophic lateral sclerosis (ALS) remains largely unknown. To this end, we studied the alteration of vimentin (a neural precursor cells marker in CNS)-containing cells (VCCs) in spinal cord during different stages of ALS used C57BL/6J G93A SOD1 transgenic mice mimicking ALS. Results showed that VCCs were mostly distributed in the ependymal zone (EZ) surrounding the central canal of spinal cord in SOD1 wild type mice; a few of VCCs were sparsely distributed in other regions. However, the number of VCCs significantly increased in the spinal cord during the onset and progression stages of ALS. They were extensively distributed in the EZ, the anterior, the lateral and the posterior horn of grey matter, particularly in the posterior horn region at the progression stage. A majority of VCCs in the anterior, the lateral and the posterior horn of grey matter (outside of EZ) generated astrocytes, but no neurons, oligodendrocytes and microgliocytes. Our results suggested that there was a potential astrocyte regenerative response to motor neuron degeneration in motor neurons-degenerated regions in the adult spinal cord during the onset and progression stages of ALS-like disease. The regenerative responses in the adult spinal cord of ALS-like mice may be a potential pathway in attempting to repair the degenerated motor neurons and restore the dysfunctional neural circuitry.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / metabolism
  • Amyotrophic Lateral Sclerosis / pathology*
  • Animals
  • Astrocytes / pathology*
  • Astrocytes / physiology*
  • Cell Differentiation / physiology
  • Cell Movement / physiology
  • Cell Proliferation / physiology
  • Disease Models, Animal
  • Disease Progression
  • Female
  • Male
  • Mice, Inbred C57BL
  • Mice, Transgenic
  • Neural Stem Cells / pathology
  • Neural Stem Cells / physiology
  • Regeneration / physiology*
  • Spinal Cord / metabolism
  • Spinal Cord / pathology*
  • Stem Cells / pathology
  • Stem Cells / physiology
  • Superoxide Dismutase / genetics
  • Superoxide Dismutase / metabolism*
  • Vimentin / metabolism*

Substances

  • Vimentin
  • SOD1 G93A protein
  • Superoxide Dismutase