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- Title
Wild-type nonneuronal cells extend survival of SOD1 mutant motor neurons in ALS mice.
- Authors
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, Jr; Julien, J-P; Goldstein, L S B; Cleveland, D W
- Abstract
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 cells. Normal motor neurons in SOD1 mutant chimeras develop aspects of ALS pathology. Most important, nonneuronal cells that do not express mutant SOD1 delay degeneration and significantly extend survival of mutant-expressing motor neurons.
- Publication
Science (New York, N.Y.), 2003, Vol 302, Issue 5642, p113
- ISSN
1095-9203
- Publication type
Journal Article
- DOI
10.1126/science.1086071