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Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis

1998; Wiley; Volume: 282; Issue: 1-2 Linguagem: Inglês

10.1002/(sici)1097-010x(199809/10)282

1097-010X

Brett M. Morrison, John H. Morrison, Jon W. Gordon,

Neurological diseases and metabolism

Journal of Experimental ZoologyVolume 282, Issue 1-2 p. 32-47 Isozymes and Biomedical Applications Superoxide dismutase and neurofilament transgenic models of amyotrophic lateral sclerosis Brett M. Morrison, Brett M. Morrison Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, New York 10029Search for more papers by this authorJohn H. Morrison, John H. Morrison Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, New York 10029 Department of Geriatrics and Adult Development, Mount Sinai School of Medicine, New York, New York 10029Search for more papers by this authorJon W. Gordon, Corresponding Author Jon W. Gordon jgordon@smtplink.mssm.edu Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Department of Geriatrics and Adult Development, Mount Sinai School of Medicine, New York, New York 10029 Department of Obstetrics/Gynecology and Reproductive Science, Mount Sinai School of Medicine, New York, New York 1002920-56 Annenberg, Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029===Search for more papers by this author Brett M. Morrison, Brett M. Morrison Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, New York 10029Search for more papers by this authorJohn H. Morrison, John H. Morrison Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Fishberg Research Center for Neurobiology, Mount Sinai School of Medicine, New York, New York 10029 Department of Geriatrics and Adult Development, Mount Sinai School of Medicine, New York, New York 10029Search for more papers by this authorJon W. Gordon, Corresponding Author Jon W. Gordon jgordon@smtplink.mssm.edu Neurobiology of Aging Laboratories, Mount Sinai School of Medicine, New York, New York 10029 Department of Geriatrics and Adult Development, Mount Sinai School of Medicine, New York, New York 10029 Department of Obstetrics/Gynecology and Reproductive Science, Mount Sinai School of Medicine, New York, New York 1002920-56 Annenberg, Mount Sinai Medical Center, One Gustave L. Levy Place, New York, NY 10029===Search for more papers by this author First published: 07 December 1998 https://doi.org/10.1002/(SICI)1097-010X(199809/10)282:1/2 3.0.CO;2-OCitations: 16AboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Abstract Amyotrophic lateral sclerosis (ALS) is a devastating neurologic disease characterized by progressive motor dysfunction that leads to paralysis and eventually death. There are numerous hypotheses for the pathogenesis of this disease, but the mechanisms of degeneration were difficult to investigate before the development of animal models. Transgenic mice with alterations in either the superoxide dismutase (SOD-1) or neurofilament genes display motor neuron pathology and deficits in motor function and, therefore, provide animal models for the study of ALS neurodegeneration. Using these animal models, as well as several in vitro models, researchers have made rapid progress during the last several years toward understanding the cause and mechanism of ALS neurodegeneration. These studies have demonstrated that motor neuron degeneration in ALS may be secondary to a number of causes, including neurofilament disruption, mutations in SOD-1, and glutamate excitotoxicity. Although each of these mechanisms can cause motor neuron degeneration by itself, studies of transgenic mice have indicated several points at which these mechanisms may interact, suggesting that they are components of one general mechanism of neurodegeneration. J. Exp. Zool. 282:32–47, 1998. © 1998 Wiley-Liss, Inc. Citing Literature Volume282, Issue1-2Special Issue: Proceedings From the 9th International Congress on Isozymes, Genes, and Gene FamiliesSeptember ‐ October 1998Pages 32-47 RelatedInformation