Document Detail

Reduced von Willebrand factor-cleaving protease levels in secondary thrombotic microangiopathies and other diseases.
MedLine Citation:
PMID:  18175284     Owner:  NLM     Status:  MEDLINE    
Thrombotic thrombocytopenic purpura (TTP) is a severe multisystem thrombotic microangiopathy (TMA). Significant advances have been made in understanding the pathogenesis of TTP since the discovery of ADAMTS-13 ( A Disintegrin And Metalloproteinase with Thrombo Spondin-1-like domains), the enzyme that regulates the size of von Willebrand factor (VWF) multimers. The inherited forms of TTP are mainly caused by a severe ADAMTS-13 deficiency, yet many aspects of the complex biological relationships between VWF-cleaving metalloproteinase and acquired TMA are still unclear. This latter issue will be critically addressed in this review article. In addition, the published literature evaluating plasma ADAMTS-13 levels in other pathologic conditions different from TMA will also be discussed.
Massimo Franchini; Martina Montagnana; Giovanni Targher; Giuseppe Lippi
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Publication Detail:
Type:  Journal Article; Review    
Journal Detail:
Title:  Seminars in thrombosis and hemostasis     Volume:  33     ISSN:  0094-6176     ISO Abbreviation:  Semin. Thromb. Hemost.     Publication Date:  2007 Nov 
Date Detail:
Created Date:  2008-01-04     Completed Date:  2008-02-27     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0431155     Medline TA:  Semin Thromb Hemost     Country:  United States    
Other Details:
Languages:  eng     Pagination:  787-97     Citation Subset:  IM    
Servizio di Immunoematologia e Trasfusione - Centro Emofilia, Azienda Ospedaliera di Verona, Verona, Italy.
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MeSH Terms
ADAM Proteins / blood*,  deficiency
Autoimmune Diseases / enzymology
Bone Marrow Transplantation
Infection / blood
Neoplasms / enzymology
Purpura, Thrombotic Thrombocytopenic / enzymology*
Thrombosis / enzymology*
von Willebrand Factor / metabolism
Reg. No./Substance:
0/von Willebrand Factor; EC 3.4.24.-/ADAM Proteins; EC 3.4.24.-/ADAMTS13 protein, human

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