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rdf:type |
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lifeskim:mentions |
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pubmed:issue |
9
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pubmed:dateCreated |
2007-7-24
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pubmed:abstractText |
1. Sickle cell disease (SCD) is an inherited disorder of haemoglobin synthesis that is associated with significant morbidity and mortality due to sequelae of episodic vaso-occlusive events: pain crises and multiorgan damage. The microvascular responses to the initiation, progression and resolution of vaso-occlusive events are consistent with an inflammatory phenotype as suggested by activation of multiple cell types, an oxidatively stressed environment and endothelial cell dysfunction. 2. Decreased anti-oxidant defences in SCD patients and mice are accompanied by activation of enzymatic (NADPH oxidase, xanthine oxidase) and non-enzymatic (sickle haemoglobin auto-oxidation) sources of reactive oxygen species. The resultant oxidative stress leads to dysfunction/activation of arteriolar and venular endothelial cells, resulting in impaired vasomotor function and blood cell-endothelial cell adhesion. 3. Changes in substrate and cofactor availability for endothelial cell nitric oxide synthase may underlie reactive oxygen- and nitrogen-induced events that contribute to SCD-induced vasculopathy. 4. The emerging role of reactive oxygen and nitrogen species in the pathogenesis of SCD provides a platform for the development of novel agents to treat this painful and lethal disease.
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pubmed:language |
eng
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pubmed:journal |
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pubmed:citationSubset |
IM
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pubmed:chemical |
http://linkedlifedata.com/resource/pubmed/chemical/Antioxidants,
http://linkedlifedata.com/resource/pubmed/chemical/Antisickling Agents,
http://linkedlifedata.com/resource/pubmed/chemical/Enzyme Inhibitors,
http://linkedlifedata.com/resource/pubmed/chemical/Hemoglobin, Sickle,
http://linkedlifedata.com/resource/pubmed/chemical/Hydroxyurea,
http://linkedlifedata.com/resource/pubmed/chemical/Iron Chelating Agents,
http://linkedlifedata.com/resource/pubmed/chemical/NADPH Oxidase,
http://linkedlifedata.com/resource/pubmed/chemical/Nitric Oxide,
http://linkedlifedata.com/resource/pubmed/chemical/Nitric Oxide Synthase,
http://linkedlifedata.com/resource/pubmed/chemical/Reactive Nitrogen Species,
http://linkedlifedata.com/resource/pubmed/chemical/Reactive Oxygen Species,
http://linkedlifedata.com/resource/pubmed/chemical/Xanthine Oxidase
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pubmed:status |
MEDLINE
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pubmed:month |
Sep
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pubmed:issn |
0305-1870
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pubmed:author |
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pubmed:issnType |
Print
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pubmed:volume |
34
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
926-32
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pubmed:dateRevised |
2007-11-15
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pubmed:meshHeading |
pubmed-meshheading:17645642-Anemia, Sickle Cell,
pubmed-meshheading:17645642-Animals,
pubmed-meshheading:17645642-Antioxidants,
pubmed-meshheading:17645642-Antisickling Agents,
pubmed-meshheading:17645642-Endothelium, Vascular,
pubmed-meshheading:17645642-Enzyme Inhibitors,
pubmed-meshheading:17645642-Erythrocyte Aggregation,
pubmed-meshheading:17645642-Hemoglobin, Sickle,
pubmed-meshheading:17645642-Hemolysis,
pubmed-meshheading:17645642-Humans,
pubmed-meshheading:17645642-Hydroxyurea,
pubmed-meshheading:17645642-Iron Chelating Agents,
pubmed-meshheading:17645642-Leukocyte Rolling,
pubmed-meshheading:17645642-NADPH Oxidase,
pubmed-meshheading:17645642-Nitric Oxide,
pubmed-meshheading:17645642-Nitric Oxide Synthase,
pubmed-meshheading:17645642-Oxidative Stress,
pubmed-meshheading:17645642-Platelet Adhesiveness,
pubmed-meshheading:17645642-Reactive Nitrogen Species,
pubmed-meshheading:17645642-Reactive Oxygen Species,
pubmed-meshheading:17645642-Vasodilation,
pubmed-meshheading:17645642-Xanthine Oxidase
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pubmed:year |
2007
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pubmed:articleTitle |
Sickle cell disease: role of reactive oxygen and nitrogen metabolites.
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pubmed:affiliation |
Department of Molecular and Cellular Physiology, Louisiana State University Health Sciences Center, Shreveport, Louisiana 71130-3932, USA.
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pubmed:publicationType |
Journal Article,
Review
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