Document Detail

Oxidative stress and disturbed glutamate transport in hereditary nucleotide repair disorders.
MedLine Citation:
PMID:  11305870     Owner:  NLM     Status:  MEDLINE    
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.
M Hayashi; M Itoh; S Araki; S Kumada; K Shioda; K Tamagawa; T Mizutani; Y Morimatsu; M Minagawa; M Oda
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of neuropathology and experimental neurology     Volume:  60     ISSN:  0022-3069     ISO Abbreviation:  J. Neuropathol. Exp. Neurol.     Publication Date:  2001 Apr 
Date Detail:
Created Date:  2001-04-17     Completed Date:  2001-04-19     Revised Date:  2004-11-17    
Medline Journal Info:
Nlm Unique ID:  2985192R     Medline TA:  J Neuropathol Exp Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  350-6     Citation Subset:  IM    
Department of Clinical Neuropathology, Tokyo Metropolitan Institute for Neuroscience, Japan.
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MeSH Terms
ATP-Binding Cassette Transporters / metabolism*
Aldehydes / metabolism
Amino Acid Transport System X-AG
Biological Transport
Cockayne Syndrome / metabolism*,  pathology
DNA Repair
Glutamic Acid / metabolism*
Glycosylation End Products, Advanced / metabolism
Neuroglia / metabolism,  pathology
Neurons / metabolism,  pathology
Oxidative Stress*
Proteins / metabolism
Tyrosine / analogs & derivatives*,  metabolism
Xeroderma Pigmentosum / metabolism*,  pathology
Reg. No./Substance:
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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