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Von Willebrand Factor and ADAMTS13 [PDF]
Arteriosclerosis, Thrombosis, and Vascular Biology, 2011 Objective— The goal of this study was to search for an association between a desintegrin-like and metalloprotease thrombospondin type 1 motif, member 13 (ADAMTS13) levels and the occurrence of preeclampsia, its characteristics ...
Alain Stepanian +2 more
exaly +2 more sources
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ADAMTS13 assays and ADAMTS13-deficient mice
Current Opinion in Hematology, 2007Thrombotic thrombocytopenic purpura can be induced by acquired or congenital deficiency of the plasma von Willebrand factor-cleaving protease, ADAMTS13. Measurement of ADAMTS13 activity is important for the diagnosis and treatment of microangiopathies including thrombotic thrombocytopenic purpura.
Toshiyuki, Miyata +4 more
openaire +2 more sources
Current Opinion in Hematology, 2002
Thrombotic thrombocytopenic purpura (TTP) has been a mysterious and deadly disease that often could be treated effectively by plasma exchange, but without real understanding of the underlying pathophysiology. Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP.
Xinglong, Zheng +2 more
openaire +2 more sources
Thrombotic thrombocytopenic purpura (TTP) has been a mysterious and deadly disease that often could be treated effectively by plasma exchange, but without real understanding of the underlying pathophysiology. Recent advances now suggest that deficiency of a specific von Willebrand factor (VWF) cleaving protease promotes tissue injury in TTP.
Xinglong, Zheng +2 more
openaire +2 more sources
Transcriptional regulation of ADAMTS13
Thrombosis and Haemostasis, 2005SummaryThe metalloproteinase ADAMTS13 cleaves VWF multimers instantaneously when they are released from endothelial cells. Absent or manifestly diminished proteolytic activity of ADAMTS13 results in the appearance and accumulation of ultralarge VWF multimers (ULVWFM) in plasma, characterised by the manifestation of Thrombotic Thrombocytopenic Purpura ...
Claus, Ralf A. +7 more
openaire +3 more sources
Blood, 2005
It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported.
Gallia G, Levy +2 more
openaire +2 more sources
It has now been 3 years since the von Willebrand factor (VWF)–cleaving protease implicated in thrombocytopenic purpura (TTP) pathogenesis was identified as ADAMTS13 (adisintegrin-like and metalloprotease with thrombospondin type 1 motif 13). More than 50 ADAMTS13 mutations resulting in familial TTP have been reported.
Gallia G, Levy +2 more
openaire +2 more sources
ADAMTS13 binds to CD36: a potential mechanism for platelet and endothelial localization of ADAMTS13
Transfusion, 2009BACKGROUND: ADAMTS13 cleaves ultralarge von Willebrand factor (VWF) and plays a significant role in vascular biology and thrombotic thrombocytopenic purpura. CD36, a transmembrane protein present on endothelial cells and platelets (PLTs), binds to thrombospondin via three thrombospondin type 1 repeats.
Amanda K, Davis +4 more
openaire +2 more sources
Variants in ADAMTS13 and Smoking Contribute to Plasma ADAMTS13 Level Variation
Blood, 2014Abstract Homeostatic processing of von Willebrand factor (VWF) in the circulation relies on proteolysis of ultra-large VWF (ULVWF) multimers by the metalloprotease ADAMTS13. Deficiency in ADAMTS13 results in Thrombotic Thrombocytopenic Purpura (TTP) due to the accumulation of thrombogenic ULVWF.
Karl C. Desch +6 more
openaire +1 more source
2015
ADAMTS13 has been identified as the von Willebrand factor-cleaving protease and is deficient in the disorder thrombotic thrombocytopenic purpura. The identification of vascular endothelium as a site of synthesis and secretion of ADAMTS13 has led to studies of the role of ADAMTS13 in angiogenesis.
Manfai Lee, George M. Rodgers
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ADAMTS13 has been identified as the von Willebrand factor-cleaving protease and is deficient in the disorder thrombotic thrombocytopenic purpura. The identification of vascular endothelium as a site of synthesis and secretion of ADAMTS13 has led to studies of the role of ADAMTS13 in angiogenesis.
Manfai Lee, George M. Rodgers
openaire +1 more source
Measurement of ADAMTS13 activity and inhibitors
Current Opinion in Hematology, 2005Acquired or congenital deficiency in the plasma von Willebrand factor-cleaving protease ADAMTS13 causes life-threatening thrombotic thrombocytopenic purpura. This condition is characterized primarily by thrombocytopenia and microangiopathic hemolytic anemia, accompanied by variable degrees of neurologic dysfunction, renal failure, and fever ...
Toshiyuki, Miyata +2 more
openaire +2 more sources
Improving on nature: redesigning ADAMTS13
Blood, 2012In this issue of Blood , Jian and coworkers report on a gain-of function variant of ADAMTS13 that is resistant to the autoantibodies responsible for acquired thrombotic thrombocytopenic purpura (TTP).[1][1] Auto-antibodies directed toward ADAMTS13 prohibit cleavage of von Willebrand factor (VWF)
Kremer Hovinga Strebel, Johanna Anna +1 more
openaire +4 more sources