Article
Transgenic mouse models of amyotrophic lateral sclerosis.
Biochimica et biophysica acta - 1 Jan 2000
Julien Jean-Pierre, Kriz Jasna
Abstract excerpt
The discovery of missense mutations in the gene coding for the Cu/Zn superoxide dismutase 1 (SOD1) in subsets of familial cases was rapidly followed by the generation of transgenic mice expressing various forms of SOD1 mutants. The mice overexpressing high levels of mutant SOD1 mRNAs do develop motor neuron disease but unraveling the mechanisms of pathogenesis has been very challenging. Studies with mouse lines...
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