Sci. Aging Knowl. Environ., 8 October 2003
Vol. 2003, Issue 40, p. or20


Wild-Type Nonneuronal Cells Extend Survival of SOD1 Mutant Motor Neurons in ALS Mice

A. M. Clement, M. D. Nguyen, E. A. Roberts, M. L. Garcia, S. Boillée, M. Rule, A. P. McMahon, W. Doucette, D. Siwek, R. J. Ferrante, R. H. Brown Jr., J.-P. Julien, L. S. B. Goldstein, and D. W. Cleveland;2003/40/or20

Abstract: Science 302, 113-117 (2003)

The most common 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.

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