Postnatal electrical and morphological abnormalities in lumbar motoneurons from transgenic mouse models of amyotrophic lateral sclerosis

Arch Ital Biol. 2007 Nov;145(3-4):311-23.


Antidromically identified lumbar motoneurons intracellularly recorded in the entire brainstem/spinal cord preparation isolated from SOD1(G85R) postnatal mice (P3-P10) were labelled with neurobiotin and fully reconstructed in 3D from serial sections in order to analyse their morphology. This staining procedure revealed differences between WT and SOD1(G85R) dendritic trees for most metric and topologic parameters analyzed. A highly complex morphology of SOD1(G85R) motoneurons dendrites (increased number of branching points and terminations) was found and the dendritic trees were longer compared to the WT motoneurons. These morphological changes observed in P8-P9 motoneurons mice occurred concomitantly with a decrease in the input resistance and gain. During electrophysiological recordings, four patterns of discharge were observed in response to ramp stimulations, that were equally distributed in WT and SOD1(G85R) motoneurons. In slice preparation, whole cell patch-clamp recordings made from developing motoneurons in SOD1(G85R) and double transgenic SOD1(G93A)/Hb9-eGFP mice showed that Riluzole, a blocker of persistent inward sodium conductance, altered the repetitive firing in a similar way for the 2 strains. These results show that the SOD1 mutations linked to familial ALS alter the development of the electrical and morphological properties of lumbar motoneurons.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Action Potentials / drug effects
  • Action Potentials / genetics
  • Amyotrophic Lateral Sclerosis / drug therapy
  • Amyotrophic Lateral Sclerosis / pathology*
  • Amyotrophic Lateral Sclerosis / physiopathology*
  • Animals
  • Animals, Newborn
  • Biotin / analogs & derivatives
  • Cell Differentiation / physiology
  • Cell Polarity / genetics
  • Cell Shape / genetics
  • Dendrites / pathology
  • Dendrites / physiology
  • Disease Models, Animal
  • Humans
  • Lumbosacral Region
  • Mice
  • Mice, Transgenic
  • Motor Neurons / pathology*
  • Motor Neurons / physiology
  • Nerve Degeneration / drug therapy
  • Nerve Degeneration / genetics
  • Nerve Degeneration / physiopathology
  • Organ Culture Techniques
  • Riluzole / pharmacology
  • Riluzole / therapeutic use
  • Sodium Channel Blockers / pharmacology
  • Sodium Channel Blockers / therapeutic use
  • Sodium Channels / drug effects
  • Sodium Channels / metabolism
  • Spinal Cord / growth & development
  • Spinal Cord / pathology*
  • Spinal Cord / physiopathology*
  • Superoxide Dismutase / genetics
  • Superoxide Dismutase-1


  • SOD1 protein, human
  • Sodium Channel Blockers
  • Sodium Channels
  • neurobiotin
  • Biotin
  • Riluzole
  • Sod1 protein, mouse
  • Superoxide Dismutase
  • Superoxide Dismutase-1