Technical Details:
Mutations in superoxide dismutase 1 cause familial forms of amyotrophic lateral sclerosis (ALS). To investigate potential mechanisms of disease pathogenesis, transgenic mice that express human superoxide dismutase 1 with the following mutations were generated: (1) a double mutation at residue 46 and 48 with histidine 46 mutated to arginine and histidine 48 mutated to glutamate; (2) a quadruple mutation where histidine 46 is mutated to arginine, histidine 48 mutated to glutamine and histidines 63 and 120 both mutated to glycine.
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This mouse model provides an important research tool for studying the pathogenesis of ALS.
