Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis.

@article{Azzouz1997ProgressiveMN,
  title={Progressive motor neuron impairment in an animal model of familial amyotrophic lateral sclerosis.},
  author={Mimoun Azzouz and Nicole Leclerc and Mark Gurney and Jean Marie Warter and Philippe Poindron and Jacqueline Borg},
  journal={Muscle & nerve},
  year={1997},
  volume={20 1},
  pages={45-51}
}
Mutations of Cu,Zn superoxide dismutase cause an autosomal dominant form of familial amyotrophic lateral sclerosis. An animal model of the disease has been produced by expressing mutant human SOD1 in transgenic mice (G93A). In order to quantify the dysfunction of the motor unit in transgenic mice, electromyographic recordings were performed during the course of the disease. The first alterations in neuromuscular function appeared between P63 and P90. The deficits became even more striking after… CONTINUE READING
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