| Pathogenesis of thrombotic thrombocytopenic purpura. | |
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MedLine Citation:
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PMID: 12901144 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The recent discovery of important molecular and genetic mechanisms of thrombotic thrombocytopenic purpura and hemolytic uremic syndrome provide an opportunity to reconstruct scientific and clinical paradigms. Acquired and congenital defects of the metalloprotease ADAMTS13 are a central feature in the pathogenesis of a major type of thrombotic microangiopathy. This and other pathogenic mechanisms can redefine the terminology of thrombotic microangiopathy. Deficient activity of ADAMTS13 suggests several possible models of microvascular thrombosis. The sporadic relationship between thrombotic microangiopathy and ADAMTS13 deficiency draws attention to other critical pathologic factors that are still poorly understood. Investigation of vascular injury and mechanisms of microvascular thrombosis remain the frontiers of investigation in thrombotic microangiopathy. |
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Authors:
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Thomas J Raife |
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Publication Detail:
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Type: Journal Article; Review |
Journal Detail:
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Title: Current hematology reports Volume: 2 ISSN: 1540-3408 ISO Abbreviation: Curr. Hematol. Rep. Publication Date: 2003 Mar |
Date Detail:
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Created Date: 2003-08-06 Completed Date: 2003-10-15 Revised Date: 2006-11-15 |
Medline Journal Info:
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Nlm Unique ID: 101151358 Medline TA: Curr Hematol Rep Country: United States |
Other Details:
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Languages: eng Pagination: 133-8 Citation Subset: IM |
Affiliation:
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Department of Pathology, C250 GH, University of Iowa Hospitals and Clinics, Iowa City, IA 52242, USA. thomas-raife@uiowa.edu |
Export Citation:
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APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
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ADAM Proteins Blood Platelets / metabolism, pathology Hemolytic-Uremic Syndrome / etiology Humans Metalloendopeptidases / deficiency Microcirculation / pathology Purpura, Thrombotic Thrombocytopenic / etiology* von Willebrand Factor / metabolism |
| Chemical | |
Reg. No./Substance:
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0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human; EC 3.4.24.-/Metalloendopeptidases |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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