Article
[Amyotrophic lateral sclerosis: recent insights from transgenic animal models with SOD1 mutations].
Rinsho shinkeigaku = Clinical neurology - 1 Nov 2004
Aoki Masashi
Abstract excerpt
Mutations in Cu/Zn superoxide dismutase (SOD1) have been linked to some familial cases of amyotrophic lateral sclerosis (ALS). In order to reproduce the different degree of toxicity to the mutant protein by mutations, we generated new transgenic mice with two mutations from which the progression of the disease in human family is rapid (L84V) or extremely slow (H46R). By comparing the two transgenic mice with...
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