| Oxidative stress and disturbed glutamate transport in hereditary nucleotide repair disorders. | |
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MedLine Citation:
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PMID: 11305870 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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Xeroderma pigmentosum group A (XPA) and Cockayne syndrome (CS) are hereditary DNA repair disorders complicated by progressive neurodegeneration. Here we immunohistochemically examine the in situ expression of materials that are produced by oxidative stress and glutamate transporters (which can contribute to prevention of glutamate neurotoxicity) in the brains of 5 autopsied patients each of XPA, CS, and control groups. All oxidative products, including nitrotyrosine, advanced glycation end product, and 4-hydroxy-2-nonenal-modified protein (HNE) were deposited in large amounts in the globus pallidus of CS patients compared to XPA patients. They were frequently recognized in the pseudocalcified foci and free minerals in the neuropil, and more rarely in foamy spheroids. In addition, the deposition of HNE was observed also in hippocampal and cerebellar dentate neurons of both CS and XPA patients. The expression of glial glutamate transporters, EAAT1 and GLT-1, was affected in the globus pallidus in 5 CS patients and 3 XPA patients. They were also altered in the cerebellar cortex in most of the CS patients. These data suggest that oxidative stress and disturbed glutamate transport may be involved in pallidal and/or cerebellar degeneration in hereditary nucleotide repair disorders. |
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Authors:
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M Hayashi; M Itoh; S Araki; S Kumada; K Shioda; K Tamagawa; T Mizutani; Y Morimatsu; M Minagawa; M Oda |
Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Journal of neuropathology and experimental neurology Volume: 60 ISSN: 0022-3069 ISO Abbreviation: J. Neuropathol. Exp. Neurol. Publication Date: 2001 Apr |
Date Detail:
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Created Date: 2001-04-17 Completed Date: 2001-04-19 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 2985192R Medline TA: J Neuropathol Exp Neurol Country: United States |
Other Details:
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Languages: eng Pagination: 350-6 Citation Subset: IM |
Affiliation:
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Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Japan. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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ATP-Binding Cassette Transporters
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metabolism* Adolescent Adult Aldehydes / metabolism Amino Acid Transport System X-AG Biological Transport Child Cockayne Syndrome / metabolism*, pathology DNA Repair Female Glutamic Acid / metabolism* Glycosylation End Products, Advanced / metabolism Humans Immunohistochemistry Male Neuroglia / metabolism, pathology Neurons / metabolism, pathology Oxidative Stress* Proteins / metabolism Tyrosine / analogs & derivatives*, metabolism Xeroderma Pigmentosum / metabolism*, pathology |
| Chemical | |
Reg. No./Substance:
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0/ATP-Binding Cassette Transporters; 0/Aldehydes; 0/Amino Acid Transport System X-AG; 0/Glycosylation End Products, Advanced; 0/Proteins; 29343-52-0/4-hydroxy-2-nonenal; 3604-79-3/3-nitrotyrosine; 55520-40-6/Tyrosine; 56-86-0/Glutamic Acid |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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