Article
Rats expressing human cytosolic copper-zinc superoxide dismutase transgenes with amyotrophic lateral sclerosis: associated mutations develop motor neuron disease.
The Journal of neuroscience : the official journal of the Society for Neuroscience - 1 Dec 2001
Nagai M, Aoki M, Miyoshi I, Kato M, Pasinelli P, Kasai N, Brown R H, Itoyama Y
Abstract excerpt
Some cases of familial amyotrophic lateral sclerosis (ALS) are caused by mutations in the gene encoding cytosolic, copper-zinc superoxide dismutase (SOD1). We report here that rats that express a human SOD1 transgene with two different ALS-associated mutations (G93A and H46R) develop striking motor neuron degeneration and paralysis. As in the human disease and transgenic ALS mice, pathological analysis...
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