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Science 3 October 2003:
Vol. 302. no. 5642, pp. 113 - 117
DOI: 10.1126/science.1086071
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Reports

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

A. M. Clement,1,3,4* M. D. Nguyen,5{dagger} E. A. Roberts,2,3 M. L. Garcia,1,3,4 S. Boillée,1,3,4 M. Rule,6 A. P. McMahon,6 W. Doucette,7 D. Siwek,8 R. J. Ferrante,8 R. H. Brown, Jr.,7 J.-P. Julien,5{ddagger} L. S. B. Goldstein,2,3 D. W. Cleveland1,3,4§

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.

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.
6 Department of Molecular and Cellular Biology, Harvard University, 16 Divinity Avenue, Cambridge, MA 02138, USA.
7 Day Neuromuscular Research Laboratory, Massachusetts General Hospital–East, Charlestown, MA 02139, USA.
8 Departments of Neurology, Pathology, and Psychiatry, Boston University School of Medicine, Bedford VA Medical Center, Geriatric Research Education Clinical Center, Bedford, MA 01730, USA.



* Present address: Institute for Physiological Chemistry and Pathobiochemistry, Johannes-Gutenberg-University, Duesbergweg 6, 55099 Mainz, Germany.

{dagger} Present address: Department of Pathology, Harvard Medical School, 200 Longwood Avenue, Boston, MA 02115, USA.

{ddagger} Present address: Centre de Recherche de l'Université Laval (CHUL), Quebec, QC, Canada G1V 4G2.

§ To whom correspondence should be addressed. E-mail: dcleveland{at}ucsd.edu

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J. Neurosci. 24, 2143-2155
   Abstract »    Full Text »    PDF »
Exacerbation of Motor Neuron Disease by Chronic Stimulation of Innate Immunity in a Mouse Model of Amyotrophic Lateral Sclerosis.
M. D. Nguyen, T. D'Aigle, G. Gowing, J.-P. Julien, and S. Rivest (2004)
J. Neurosci. 24, 1340-1349
   Abstract »    Full Text »    PDF »
Healthy Nonneuronal Cells Protect Motor Neurons in ALS Mice.
(2004)
Journal Watch Neurology 2004, 5
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Science. ISSN 0036-8075 (print), 1095-9203 (online)