Source:http://linkedlifedata.com/resource/pubmed/id/15497097
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Predicate | Object |
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rdf:type | |
lifeskim:mentions | |
pubmed:issue |
5
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pubmed:dateCreated |
2004-10-21
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pubmed:abstractText |
Thrombotic thrombocytopenic purpura (TTP) is characterized by thrombocytopenia and microangiopathic hemolysis. Unlike the typical hemolytic uremic syndrome (HUS), which follows infection with shiga toxin-producing microorganisms, most cases of TTP do not have an obvious etiology. Recent studies revealed that a plasma zinc metalloprotease ADAMTS (a disintegrin and metalloprotease with thrombospondin type 1 motif) 13 cleaves von Willebrand factor in a shear-dependent manner. Deficiency of ADAMTS13, due to autoimmune inhibitors of the protease or genetic mutation in the ADAMTS13 gene, results in a propensity to the development of von Willebrand factor-platelet aggregation and intravascular thrombosis characteristic of TTP. The identification of the molecular defect in TTP raises the prospect that this hitherto mysterious disorder will be managed with a more rationally designed strategy in the near future.
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pubmed:grant | |
pubmed:language |
eng
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pubmed:journal | |
pubmed:citationSubset |
IM
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pubmed:chemical | |
pubmed:status |
MEDLINE
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pubmed:month |
Oct
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pubmed:issn |
0094-6176
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pubmed:author | |
pubmed:issnType |
Print
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pubmed:volume |
30
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pubmed:owner |
NLM
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pubmed:authorsComplete |
Y
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pubmed:pagination |
549-57
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pubmed:dateRevised |
2011-8-1
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pubmed:meshHeading |
pubmed-meshheading:15497097-ADAM Proteins,
pubmed-meshheading:15497097-Blood Platelets,
pubmed-meshheading:15497097-Cloning, Molecular,
pubmed-meshheading:15497097-Hemolytic-Uremic Syndrome,
pubmed-meshheading:15497097-Humans,
pubmed-meshheading:15497097-Metalloendopeptidases,
pubmed-meshheading:15497097-Mutation,
pubmed-meshheading:15497097-Purpura, Thrombotic Thrombocytopenic,
pubmed-meshheading:15497097-von Willebrand Factor
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pubmed:year |
2004
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pubmed:articleTitle |
Molecular mechanisms in thrombotic thrombocytopenic purpura.
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pubmed:affiliation |
Albert Einstein College of Medicine and Montefiore Medical Center, Bronx, New York, USA. htsai@montefiore.org
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pubmed:publicationType |
Journal Article,
Research Support, U.S. Gov't, P.H.S.,
Review
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