Results 191 to 200 of about 13,808 (210)

ADAMTS13 Activity: Screening Test Protocol

2023
Accurate estimation of ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level is necessary for diagnosis and management of thrombotic microangiopathies (TMA). In particular, it permits distinction between thrombotic thrombocytopenic purpura (TTP) and other TMAs, prompting disorder appropriate ...
Gary W, Moore, Margaret, Griffiths, Nikolaus B, Binder   +2 more
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ADAMTS13's tail tale

Blood, 2009
In mice, a long form and a short form of the VWF-cleaving protease ADAMTS13 have been identified, the latter lacking the 4 distal carboxyl-terminal domains. While these are not strictly required for regulating normal size distribution of VWF multimers, in this issue of Blood, Banno and colleagues reveal the role of these domains in down-regulating ...
Karen, Vanhoorelbeke, Hendrik B, Feys, Simon F, De Meyer   +2 more
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Transcriptional regulation of ADAMTS13

Thrombosis and Haemostasis, 2005
SummaryThe 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., Bockmeyer, Clemens L., Kentouche, Karim, Sieber, Matthias W., Oberle, Volker, Kaufmann, Roland, Deigner, Hans-Peter, Lösche, Wolfgang   +7 more
openaire   +3 more sources

ADAMTS13 Antibody and Inhibitor Assays

2023
A finding of an ADAMTS13 (a disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13) activity level of
Gary W, Moore, Helga, Vetr, Nikolaus B, Binder   +2 more
openaire   +2 more sources

ADAMTS13 in Health and Disease

Acta Haematologica, 2009
We raised a set of monoclonal antibodies (mAb) against recombinant human ADAMTS13, constructed the first antigen test fully based on mAbs and compared ADAMTS13 antigen and enzymatic activity levels in a large set of plasma samples collected from different patients and healthy controls.
Hendrik B, Feys, Hans, Deckmyn, Karen, Vanhoorelbeke   +2 more
openaire   +2 more sources

ADAMTS13: origins, applications, and prospects

Transfusion, 2018
ADAMTS13 is an enzyme that acts by cleaving prothrombotic von Willebrand factor (VWF) multimers from the vasculature in a highly regulated manner. In pathologic states such as thrombotic thrombocytopenic purpura (TTP) and other thrombotic microangiopathies (TMAs), VWF can bind to the endothelium and form large multimers. As the anchored VWF chains grow,
William E. Plautz, Jay S. Raval, Mitchell R. Dyer, Marian A. Rollins‐Raval, Brian S. Zuckerbraun, Matthew D. Neal   +5 more
openaire   +2 more sources

Improving on nature: redesigning ADAMTS13

Blood, 2012
In 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)
Hovinga, Johanna A. Kremer, Voorberg, Jan   +1 more
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The emerging concept of residual ADAMTS13 activity in ADAMTS13-deficient thrombotic thrombocytopenic purpura

Blood Reviews, 2013
Thrombotic thrombocytopenic purpura (TTP) is a rare, life-threatening disease characterized by acute episodes of widespread microvascular thrombosis. The discovery that the plasmatic activity of the von Willebrand factor cleaving protease, ADAMTS13, is severely deficient in patients with TTP partially clarified the pathophysiology of the disease ...
L. A. Lotta, H. M. Wu, K. M. Musallam, F. Peyvandi   +3 more
openaire   +3 more sources

Variants in ADAMTS13 and Smoking Contribute to Plasma ADAMTS13 Level Variation

Blood, 2014
Abstract 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, Qianyi Ma, Ayse Bilge Ozel, Beth M McGee, David R Siemieniak, Jun Z Li, David Ginsburg   +6 more
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Hemoglobin versus ADAMTS13

Blood, 2005
Comment on Studt et al, page [542][1] Free hemoglobin inhibits ADAMTS13 activity in vitro, demonstrating a potential source of artifact affecting ADAMTS13 activity assays and inhibitor screens. Deficiency of the ADAMTS13 metalloprotease causes thrombotic thrombocytopenic purpura (TTP).
openaire   +1 more source