Amyotrophic Lateral Sclerosis (ALS) Studies

Transgenic SOD1 (superoxide dismutase 1) mice express a G93A mutant form of human SOD1. SOD1 mice (TgN-SOD1-G93A-1Gur) exhibit a phenotype similar to amyotrophic lateral sclerosis (ALS) in humans, an age-related rapidly progressive decline of motor functions accompanied by degenerative changes of motoneurons within the spinal cord, brain stem and neocortex.

Charles River has obtained the license from Northwestern University/Harvard/MIT to utilize this transgenic model for contract efficacy studies. All characterization and analytical methods used with the model are performed in our proprietary labs by our own staff of scientists and technicians.

  • Body weight
  • Kaplan-Meyer survival plot
  • Motor deficits
    • Rotarod
    • Grip strength
    • Clinical scoring for disease state
    • Open field test
      • Distance traveled
      • Rearing frequency
  • MRI for neurodegeneration in the brain stem
  • MRS for metabolic changes in the brain stem
  • Motor neuron counts

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