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

Author:

Clement A. M.12345,Nguyen M. D.12345,Roberts E. A.12345,Garcia M. L.12345,Boillée S.12345,Rule M.12345,McMahon A. P.12345,Doucette W.12345,Siwek D.12345,Ferrante R. J.12345,Brown R. H.12345,Julien J.-P.12345,Goldstein L. S. B.12345,Cleveland D. W.12345

Affiliation:

1. Ludwig Institute for Cancer Research, University of California, 9500 Gilman Drive, La Jolla, CA 92093–0670, USA.

2. Howard Hughes Medical Institute, University of California, 9500 Gilman Drive, La Jolla, CA 92093–0670, USA.

3. Department of Cellular and Molecular Medicine, University of California, 9500 Gilman Drive, La Jolla, CA 92093–0670, USA.

4. Department of Neurosciences, University of California, 9500 Gilman Drive, La Jolla, CA 92093–0670, USA.

5. Centre for Research in Neurosciences, Research Institute of the McGill University Health Care Centre, Montreal General Hospital, 1650 Cedar Avenue, Montreal, Quebec, Canada H3G 1A4.

Abstract

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.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

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