Results 11 to 20 of about 8,766 (203)

Hereditary Thrombotic Thrombocytopenic Purpura Associated With Recurrent Strokes and Prominent Nervous System Involvement in a Young Chinese Female. [PDF]

Clin Case Rep
ABSTRACT Hereditary thrombotic thrombocytopenic purpura (TTP) is a rare autosomal recessive inherited disease caused by an ADAMTS1 gene mutation, resulting in absence or severe deficiency of plasma ADAMTS13 activity. The common causes include infection, inflammation, or pregnancy.
Liu W, Wu J, Ming X, Zhang Q, Zhou D, Zheng R, Zhou M, Shang Z, Chen L, Zhu X, Xiao Y.   +10 more
europepmc   +2 more sources

Improvement of recombinant ADAMTS13 production through a more optimal signal peptide or an N‐terminal fusion protein

Journal of Thrombosis and Haemostasis, 2022
Recombinant human ADAMTS13 (rADAMTS13) is a key protein in fundamental research for investigating its mode of action and the pathophysiology of thrombotic thrombocytopenic purpura (TTP). However, the expression of rADAMTS13 is quite low in mammalian cells, which makes the production of the protein time-consuming and labor-intensive.We aimed at ...
Kadri Kangro, Elien Roose, Charlotte Dekimpe, Aline Vandenbulcke, Nuno A.G. Graça, Jan Voorberg, Mart Ustav, Andres Männik, Karen Vanhoorelbeke   +8 more
openaire   +3 more sources

Pathogen-reduced plasma, cryoprecipitate reduced for therapeutic plasma exchange. [PDF]

Transfusion
Abstract Background Therapeutic plasma exchange (TPE) for thrombotic thrombocytopenic purpura (TTP) and auto‐immune disorders involves repeated patient exposure to allogenic plasma with the risk of transfusion‐transmitted infection (TTI).
Tupin F, Mouriaux C, Hechler B, Kaastrup K, Yegneswaran S, Corash L, Mangin PH.   +6 more
europepmc   +2 more sources

Local Elongation of Endothelial Cell-anchored von Willebrand Factor Strings Precedes ADAMTS13 Protein-mediated Proteolysis [PDF]

Journal of Biological Chemistry, 2011
Platelet-decorated von Willebrand factor (VWF) strings anchored to the endothelial surface are rapidly cleaved by ADAMTS13. Individual VWF string characteristics such as number, location, and auxiliary features of the ADAMTS13 cleavage sites were explored here using imaging and computing software.
De Ceunynck, Karen, Rocha, Susana, Feys, Hendrik B., De Meyer, Simon, Uji-I, Hiroshi, Deckmyn, Hans, Hofkens, Johan, Vanhoorelbeke, Karen   +7 more
openaire   +3 more sources

Decreased protein C activity, lower ADAMTS13 antigen and free protein S levels accompanied by unchanged thrombin generation potential in hospitalized COVID-19 patients

Thrombosis Research, 2023
COVID-19 is associated with an increased thromboembolic risk. However, the mechanisms triggering clot formation in those patients remain unknown.In 118 adult Caucasian severe but non-critically ill COVID-19 patients (median age 58 years; 73 % men) and 46 controls, we analyzed in vitro plasma thrombin generation profile (calibrated automated thrombogram
Wójcik, Krzysztof, Bazan-Socha, Stanisława, Celejewska-Wójcik, Natalia, Górka, Karolina, Lichołai, Sabina, Polok, Kamil, Stachura, Tomasz, Zaręba, Lech, Dziedzic, Radosław, Gradzikiewicz, Ada, Sanak, Marek, Musiał, Jacek, Sładek, Krzysztof, Iwaniec, Teresa   +13 more
openaire   +3 more sources

Escherichia coli-derived von Willebrand factor-A2 domain fluorescence/Förster resonance energy transfer proteins that quantify ADAMTS13 activity [PDF]

Analytical Biochemistry, 2011
The cleavage of the A2 domain of von Willebrand factor (VWF) by the metalloprotease ADAMTS13 regulates VWF size and platelet thrombosis rates. Reduction or inhibition of this enzyme activity leads to thrombotic thrombocytopenic purpura (TTP). We generated a set of novel molecules called VWF-A2 FRET (fluorescence/Förster resonance energy transfer ...
Kannayakanahalli M, Dayananda, Shobhit, Gogia, Sriram, Neelamegham   +2 more
openaire   +2 more sources

S109 Adamts13 protein levels are decreased in chronic thromboembolic pulmonary hypertension and implicated in its pathobiology [PDF]

Advances in understanding chronic thrombo-embolic disease and pulmonary hypertension, 2017
Introduction Chronic thromboembolic pulmonary hypertension (CTEPH) Results from failure of thrombus resolution following acute pulmonary embolism. Abnormalities in haemostasis are implicated in the pathobiology, including elevated levels of von Willebrand factor (VWF), which is normally regulated by ADAMTS13.
M Newnham, K South, M Bleda, J Cannon, S Gräf, C Hadinnapola, K Sheares, D Taboada, MR Wilkins, J Wharton, J Pepke-Zaba, M Laffan, DA Lane, M Toshner, NW Morrell   +14 more
openaire   +1 more source

ADAMTS13 inhibits H2O2-induced human venous endothelial cell injury to attenuate deep-vein thrombosis by blocking the p38/ERK signaling pathway

Chinese Journal of Physiology, 2023
Deep vein thrombosis (DVT) is a common complication in hematologic malignancies and immunologic disorders. Endothelial cell injury and dysfunction comprise the critical contributor for the development of DVT.
Guangfeng Zheng, Qiang Zhang, Chuanyong Li, Weijian Fan, Zhichang Pan, Yuting Zhou, Yan Chen, Jianjie Rong   +7 more
doaj   +1 more source

Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure. [PDF]

, 2009
Decreased activity of ADAMTS13, the von Willebrand factor (VWF) cleaving protease, was recently reported in cardiovascular diseases and in hepatic failure.
Borgulya, Gábor, Cervenak, László, Förhécz, Zsolt, Gombos, Tímea, Hársfalvi, Jolán, Jánoskuti, Lívia, Kunde, Jan, Makó, Veronika, Papassotiriou, Jana, Pozsonyi, Zoltán, Prohászka, Zoltán   +10 more
core   +1 more source

Probing ADAMTS13 substrate specificity using phage display. [PDF]

PLoS ONE, 2015
Von Willebrand factor (VWF) is a large, multimeric protein that regulates hemostasis by tethering platelets to the subendothelial matrix at sites of vascular damage.
Karl C Desch, Colin Kretz, Andrew Yee, Robert Gildersleeve, Kristin Metzger, Nidhi Agrawal, Jane Cheng, David Ginsburg   +7 more
doaj   +1 more source