A simple test to monitor the motor dysfunction in a transgenic mouse model of amyotrophic lateral sclerosis

J Psychiatry Neurosci. 1995 Jan;20(1):80-2.


We reported recently that transgenic mice overexpressing human neurofilament heavy (NF-H) proteins develop a progressive neurological disorder with pathological features resembling those found in amyotrophic lateral sclerosis (ALS) (Côté et al 1993). A simple behavioral test, the grasping ability, has been used here for evaluating the motor dysfunction during aging of NF-H transgenic mice. Transgenic mice overexpressing NF-H proteins are normal at birth but they progressively fail to uphold their weight when tested for their grasping ability. The deficits in motor function during aging correlate with a progressive disruption of peripheral nerve function as evidenced by the atrophy and degeneration of distal axons.

MeSH terms

  • Amyotrophic Lateral Sclerosis / genetics*
  • Amyotrophic Lateral Sclerosis / pathology
  • Animals
  • Axons / pathology
  • Female
  • Male
  • Mice
  • Mice, Transgenic
  • Motor Neurons / pathology
  • Motor Skills / physiology*
  • Neurofilament Proteins / genetics
  • Peripheral Nerves / pathology


  • Neurofilament Proteins
  • neurofilament protein H