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

1 Jan 1997

Abstract excerpt

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...

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PMID: 8995582
DOI: 10.1002/(sici)1097-4598(199701)20:1<45::aid-mus6>3.0.co;2-h

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