Results 91 to 100 of about 447 (133)

Assays of ADAMTS-13 activity

Seminars in Hematology, 2004
Various assays for determination of ADAMTS-13 activity in plasma have been developed, all comprising two steps. The first step consists of proteolyzing a substrate by ADAMTS-13. Substrates were either exogenous von Willebrand factor (VWF) (purified from human plasma concentrates or recombinant VWF [rVWF]), purified VWF fragments, or endogenous VWF ...
Agnès, Veyradier, Jean-Pierre, Girma
openaire   +2 more sources

Severe ADAMTS-13 deficiency in childhood

Seminars in Hematology, 2004
Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathic disorder with high morbidity and significant mortality. The primary form of TTP is caused by severe deficiency, acquired or hereditary, of the von Willebrand factor cleaving protease (VWF-CP), ADAMTS-13.
Reinhard, Schneppenheim, Ulrich, Budde, Wolf, Hassenpflug, Tobias, Obser   +3 more
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Conformational quiescence of ADAMTS‐13 prevents proteolytic promiscuity [PDF]

Journal of Thrombosis and Haemostasis, 2016
Essentials Recently, ADAMTS-13 has been shown to undergo substrate induced conformation activation. Conformational quiescence of ADAMTS-13 may serve to prevent off-target proteolysis in plasma. Conformationally active ADAMTS-13 variants are capable of proteolysing the Aα chain of fibrinogen. This should be considered as ADAMTS-13 variants are developed
D A Lane
exaly   +5 more sources

ADAMTS‐13 assays in thrombotic thrombocytopenic purpura

Journal of Thrombosis and Haemostasis, 2010
ADAMTS-13, the thirteenth member of the ADAMTS (A Disintegrin And Metalloprotease with Thrombo-Spondin 1 repeats) family, is the plasma metalloprotease responsible for regulating the multimeric structure of VWF. In congenital or acquired deficiency it is actively involved in the pathophysiology of thrombotic thrombocytopenic purpura (TTP), a rare but ...
F, Peyvandi, R, Palla, L A, Lotta, I, Mackie, M A, Scully, S J, Machin   +5 more
openaire   +2 more sources

VWF73: a substrate for ADAMTS-13

Blood, 2004
![Figure][1] Recognition that a deficiency in the metalloprotease ADAMTS-13 leads to thrombotic thrombocytopenic purpura (TTP) is an important development in understanding the pathogenesis of this disorder.
openaire   +1 more source

Human endothelial cells synthesize and release ADAMTS-13

Journal of Thrombosis and Haemostasis, 2006
Hepatic stellate cells have been considered to be a primary source for human plasma ADAMTS-13, the von Willebrand factor (VWF)-cleaving metalloprotease. In this study, ADAMTS-13 antigen was detected by immunofluorescence in both venous (HUVECs) and arterial endothelial cells (HUAECs) using both polyclonal antibodies made against peptides found in ...
J-F Dong
exaly   +3 more sources

A multi‐center evaluation of TECHNOSCREEN® ADAMTS‐13 activity assay as a screening tool for detecting deficiency of ADAMTS‐13

Journal of Thrombosis and Haemostasis, 2020
Quantifying A disintegrin-like and metalloprotease with thrombospondin type 1 motif, member 13 (ADAMTS-13) activity enhances thrombotic thrombocytopenic purpura (TTP) diagnosis but most assays are time consuming, technically demanding, and mainly available in reference centers.Evaluate a simple, semiquantitative ADAMTS-13 activity screening test for ...
Gary W, Moore, Daniëlle, Meijer, Margaret, Griffiths, Lucy, Rushen, Alice, Brown, Ulrich, Budde, Rita, Dittmer, Barbara, Schocke, Anja, Leyte, Sabine, Geiter, Anneke, Moes, Jacqueline A, Cutler, Nikolaus B, Binder   +12 more
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A child with specific ADAMTS-13 gene defect.

Pediatric Nephrology, 2010
A 4 year old child was admitted in Clinical hospital after recidivant episodes of haemolytic anaemia and thrombocytopenia registrated in early childhood, first recognized in a regional hospital in the maternity ward. The area of the highest incidence of the disease in our Continent is found in Northern Europe. Our boy is the first genetically confirmed
Batinić, Danica, Milošević, Danko, Vrljičak, Kristina   +2 more
openaire   +4 more sources

Localization and Function of Platelet ADAMTS-13

Blood, 2005
Abstract The haemostatic activity of von Willebrand factor (VWF) is strongly dependent on its multimeric size, with the highest activity in ‘unusually large’ multimers (ULVWF) secreted from endothelial cells. The multimeric size is regulated by a plasma metalloprotease, ADAMTS-13.
F. Peyvandi, I. Garagiola, A. Artoni, R. Lombardi, R. Palla, R. De Cristofaro, P.M. Mannucci, S. Lavoretano, C. Valsecchi   +8 more
openaire   +3 more sources

Another effect of desmopressin in ADAMTS-13

Blood, 2003
The adhesive glycoprotein von Willebrand factor (VWF) supports platelet interactions and stabilizes coagulation factor VIII (FVIII). Under high shear stress conditions such as those occurring in arterioles and in large stenotic arteries, plasma VWF changes from a globular into a ...
openaire   +1 more source