| Mutant superoxide dismutase-1-linked familial amyotrophic lateral sclerosis: molecular mechanisms of neuronal death and protection. | |
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MedLine Citation:
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PMID: 9348345 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Mutations in human Cu/Zn superoxide dismutase-1 (SOD) cause approximately 20% of cases of familial amyotrophic lateral sclerosis (FALS). We investigated the mechanism of mutant SOD-induced neuronal degeneration by expressing wild-type and mutant SODs in neuronal cells by means of infection with replication-deficient recombinant adenoviruses. Expression of two FALS-related mutant SODs (A4V and V148G) caused death of differentiated PC12 cells, superior cervical ganglion neurons, and hippocampal pyramidal neurons. Cell death included many features typical of apoptosis. Death could be prevented by copper (Cu2+) chelators, Bcl-2, glutathione, vitamin E, and inhibitors of caspases. Mutant SOD-expressing PC12 cells had higher rates of superoxide (O2-) production under a variety of conditions. The results support the hypothesis that mutant SOD induced-neurodegeneration is associated with disturbances of neuronal free radical homeostasis. |
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Authors:
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G D Ghadge; J P Lee; V P Bindokas; J Jordan; L Ma; R J Miller; R P Roos |
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Publication Detail:
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Type: Journal Article; Research Support, Non-U.S. Gov't; Research Support, U.S. Gov't, P.H.S. |
Journal Detail:
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Title: The Journal of neuroscience : the official journal of the Society for Neuroscience Volume: 17 ISSN: 0270-6474 ISO Abbreviation: J. Neurosci. Publication Date: 1997 Nov |
Date Detail:
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Created Date: 1997-12-08 Completed Date: 1997-12-08 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 8102140 Medline TA: J Neurosci Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 8756-66 Citation Subset: IM |
Affiliation:
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Department of Neurology, The University of Chicago, Chicago, Illinois 60637, USA. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adenoviridae
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genetics Amyotrophic Lateral Sclerosis / enzymology*, genetics* Animals Antioxidants / pharmacology Apoptosis / genetics Cell Survival / physiology Chelating Agents / pharmacology Cricetinae Family Health Free Radicals / metabolism Gene Expression Regulation, Enzymologic Gene Expression Regulation, Viral Humans Kidney / cytology Neurons / cytology*, drug effects, enzymology Oxidative Stress / physiology PC12 Cells Rats Recombinant Proteins / metabolism Superoxide Dismutase / genetics* Superoxides / metabolism Transfection |
| Chemical | |
Reg. No./Substance:
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0/Antioxidants; 0/Chelating Agents; 0/Free Radicals; 0/Recombinant Proteins; 11062-77-4/Superoxides; EC 1.15.1.1/Superoxide Dismutase |
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