| Therapeutic effects of coenzyme Q10 and remacemide in transgenic mouse models of Huntington's disease. | |
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MedLine Citation:
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PMID: 11880489 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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There is substantial evidence that bioenergetic defects and excitotoxicity may play a role in the pathogenesis of Huntington's disease (HD). Potential therapeutic strategies for neurodegenerative diseases in which there is reduced energy metabolism and NMDA-mediated excitotoxicity are the administration of the mitochondrial cofactor coenzyme Q10 and the NMDA antagonist remacemide. We found that oral administration of either coenzyme Q10 or remacemide significantly extended survival and delayed the development of motor deficits, weight loss, cerebral atrophy, and neuronal intranuclear inclusions in the R6/2 transgenic mouse model of HD. The combined treatment, using coenzyme Q10 and remacemide together, was more efficacious than either compound alone, resulting in an approximately 32 and 17% increase in survival in the R6/2 and N171-82Q mice, respectively. Magnetic resonance imaging showed that combined treatment significantly attenuated ventricular enlargement in vivo. These studies further implicate defective energy metabolism and excitotoxicity in the R6/2 and N171-82Q transgenic mouse models of HD and are of interest in comparison with the outcome of a recent clinical trial examining coenzyme Q10 and remacemide in HD patients. |
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Authors:
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Robert J Ferrante; Ole A Andreassen; Alpaslan Dedeoglu; Kimberly L Ferrante; Bruce G Jenkins; Steven M Hersch; M Flint Beal |
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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, Non-P.H.S.; 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: 22 ISSN: 1529-2401 ISO Abbreviation: J. Neurosci. Publication Date: 2002 Mar |
Date Detail:
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Created Date: 2002-03-06 Completed Date: 2002-03-22 Revised Date: 2012-07-11 |
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: 1592-9 Citation Subset: IM |
Affiliation:
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Geriatric Research Education and Clinical Center, Bedford Veterans Administration Medical Center, Bedford, Massachusetts 01730, USA. rjferr@bu.edu |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Acetamides
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therapeutic use* Administration, Oral Animals Behavior, Animal / drug effects Body Weight / drug effects Brain / drug effects, pathology Cerebral Ventricles / drug effects, pathology Coenzymes Disease Models, Animal Disease Progression Drug Evaluation, Preclinical Drug Synergism Female Humans Huntington Disease / drug therapy*, genetics, pathology Magnetic Resonance Imaging Male Mice Mice, Transgenic Motor Activity / drug effects Nerve Tissue Proteins / genetics Nuclear Proteins / genetics Organ Size / drug effects Survival Rate Treatment Outcome Ubiquinone / analogs & derivatives*, therapeutic use* |
| Grant Support | |
ID/Acronym/Agency:
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AG12992/AG/NIA NIH HHS; AG13846/AG/NIA NIH HHS; AT00613/AT/NCCAM NIH HHS; NS35255/NS/NINDS NIH HHS; NS37102/NS/NINDS NIH HHS; NS38180/NS/NINDS NIH HHS |
| Chemical | |
Reg. No./Substance:
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0/Acetamides; 0/Coenzymes; 0/HTT protein, human; 0/Hdh protein, mouse; 0/Nerve Tissue Proteins; 0/Nuclear Proteins; 128298-28-2/remacemide; 1339-63-5/Ubiquinone; 303-98-0/coenzyme Q10 |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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