Article
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
Science (New York, N.Y.) - 3 Oct 2003
Clement A M, Nguyen M D, Roberts E A, Garcia M L, Boillée S, Rule M, McMahon A P, Doucette W, Siwek D, Ferrante R J, Brown R H, Julien J-P, Goldstein L S B, Cleveland D W
Abstract excerpt
The most common inherited [correct] form of amyotrophic lateral sclerosis (ALS), a neurodegenerative disease affecting adult motor neurons, is caused by dominant mutations in the ubiquitously expressed Cu-Zn superoxide dismutase (SOD1). In chimeric mice that are mixtures of normal and SOD1 mutant-expressing cells, toxicity to motor neurons is shown to require damage from mutant SOD1 acting within nonneuronal...
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