Ultrastructural diversity of inclusions and aggregations in the lumbar spinal cord of SOD1-G93A transgenic mice

Brain Res. 2010 Sep 24;1353:234-44. doi: 10.1016/j.brainres.2010.07.025. Epub 2010 Jul 15.

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by selective motor neuron death. We report the characteristics of ultrastructural pathological changes of inclusions and aggregations in the neuronal axons, glial cells and ventral roots of lumbar spinal cord in SOD1-G93A transgenic mice using light and electron transmission microscope at different stages of disease. The most noteworthy is that mutant SOD1 accumulations in the cytoplasm of motor neurons precede the numerous inclusions. Inclusions manifested differently according to the specified locations. This study provided further information to the previous reports about pathological changes of ALS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics
  • Amyotrophic Lateral Sclerosis / pathology*
  • Animals
  • Astrocytes / pathology
  • Astrocytes / ultrastructure
  • Disease Models, Animal
  • Humans
  • Lumbosacral Region / pathology
  • Mice
  • Mice, Transgenic
  • Microscopy, Electron, Transmission / methods
  • Neurons / pathology
  • Neurons / ultrastructure
  • Spinal Cord / pathology*
  • Spinal Cord / ultrastructure*
  • Superoxide Dismutase / genetics

Substances

  • SOD1 G93A protein
  • Superoxide Dismutase