Results 21 to 30 of about 15,262 (192)

Genetic variants in ADAMTS13 as well as smoking are major determinants of plasma ADAMTS13 levels

Blood Advances, 2017
: The metalloprotease ADAMTS13 cleaves von Willebrand factor (VWF) in circulating blood, limiting the size of VWF multimers and regulating VWF activity. Abnormal regulation of VWF contributes to bleeding and to thrombotic disorders.
Qianyi Ma, Paula M. Jacobi, Brian T. Emmer, Colin A. Kretz, Ayse Bilge Ozel, Beth McGee, Chava Kimchi-Sarfaty, David Ginsburg, Jun Z. Li, Karl C. Desch   +9 more
doaj   +1 more source

Infection frequently triggers thrombotic microangiopathy in patients with preexisting risk factors : a single-institution experience [PDF]

, 2010
Thrombotic microangiopathies are rare conditions characterized by microangiopathic hemolytic anemia, microthrombi, and multiorgan insult. The disorders, which include hemolytic uremic syndrome and thrombotic thrombocytopenic purpura, are often acute and ...
Catlow, Jamie, Douglas, Kenneth W., Green, Rachel, Pollock, Kevin G.J., Young, David   +4 more
core   +1 more source

vWF/ADAMTS13 is associated with on-aspirin residual platelet reactivity and clinical outcome in patients with stable coronary artery disease

Thrombosis Journal, 2017
Background The mechanisms behind residual platelet reactivity (RPR) despite aspirin treatment are not established. It has been shown that coronary artery disease (CAD) patients with high on-aspirin RPR have elevated levels of von Willebrand factor (vWF).
Ellen M. K. Warlo, Alf-Åge R. Pettersen, Harald Arnesen, Ingebjørg Seljeflot   +3 more
doaj   +1 more source

Removal of the C-Terminal Domains of ADAMTS13 by Activated Coagulation Factor XI induces Platelet Adhesion on Endothelial Cells under Flow Conditions

Frontiers in Medicine, 2017
Platelet recruitment to sites of vascular injury is mediated by von Willebrand factor (VWF). The shear-induced unraveling of ultra-large VWF multimers causes the formation of a “stringlike” conformation, which rapidly recruits platelets from the ...
Kathleen S. Garland, Kathleen S. Garland, Stéphanie E. Reitsma, Stéphanie E. Reitsma, Toshiaki Shirai, Jevgenia Zilberman-Rudenko, Erik I. Tucker, Erik I. Tucker, David Gailani, András Gruber, András Gruber, Owen J. T. McCarty, Cristina Puy   +12 more
doaj   +1 more source

Predictive features of severe acquired ADAMTS13 deficiency in idiopathic thrombotic microangiopathies: the French TMA reference center experience. [PDF]

PLoS ONE, 2010
Severe ADAMTS13 deficiency occurs in 13% to 75% of thrombotic microangiopathies (TMA). In this context, the early identification of a severe, antibody-mediated, ADAMTS13 deficiency may allow to start targeted therapies such as B-lymphocytes-depleting ...
Paul Coppo, Michael Schwarzinger, Marc Buffet, Alain Wynckel, Karine Clabault, Claire Presne, Pascale Poullin, Sandrine Malot, Philippe Vanhille, Elie Azoulay, Lionel Galicier, Virginie Lemiale, Jean-Paul Mira, Christophe Ridel, Eric Rondeau, Jacques Pourrat, Stéphane Girault, Dominique Bordessoule, Samir Saheb, Michel Ramakers, Mohamed Hamidou, Jean-Paul Vernant, Bertrand Guidet, Martine Wolf, Agnès Veyradier, French Reference Center for Thrombotic Microangiopathies   +25 more
doaj   +1 more source

Perinatal Gene Transfer to the Liver [PDF]

, 2011
The liver acts as a host to many functions hence raising the possibility that any one may be compromised by a single gene defect. Inherited or de novo mutations in these genes may result in relatively mild diseases or be so devastating that death within
Buckley, SM, Chan, JK, Howe, SJ, McKay, TR, Rahim, AA, Waddington, SN, Ward, NJ   +6 more
core   +1 more source

ADAMTS13 ameliorates inflammatory responses in experimental autoimmune encephalomyelitis

Journal of Neuroinflammation, 2020
Background ADAMTS13 (a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13) plays a vital role in preventing microvascular thrombosis and inflammation.
Kaili Lu, Lan Liu, Xiaofeng Xu, Fei Zhao, Jiangshan Deng, Xin Tang, Xiuzhe Wang, Bing-Qiao Zhao, Xiaojie Zhang, Yuwu Zhao   +9 more
doaj   +1 more source

Reduced ADAMTS13 levels in patients with acute and chronic cerebrovascular disease. [PDF]

PLoS ONE, 2017
Von Willebrand Factor (VWF) plays a major role in thrombosis and hemostasis and its thrombogenicity is controlled by ADAMTS13. Whereas increasing evidence shows a clear association between VWF levels and acute ischemic stroke, little is known about a ...
Frederik Denorme, Peter Kraft, Inge Pareyn, Christiane Drechsler, Hans Deckmyn, Karen Vanhoorelbeke, Christoph Kleinschnitz, Simon F De Meyer   +7 more
doaj   +1 more source

Evidence for a role of anti-ADAMTS13 autoantibodies despite normal ADAMTS13 activity in recurrent thrombotic thrombocytopenic purpura

Haematologica, 2012
Background Severe ADAMTS13 deficiency is a critical component of the pathogenesis of idiopathic thrombotic thrombocytopenic purpura but is found only in about 60% of patients clinically diagnosed with this disease.Design and Methods Over a period of 8 ...
Rahel Froehlich-Zahnd, James N. George, Sara K. Vesely, Deirdra R. Terrell, Khatira Aboulfatova, Jing-Fei Dong, Brenda M. Luken, Jan Voorberg, Ulrich Budde, Irmela Sulzer, Bernhard Lämmle, Johanna A. Kremer Hovinga   +11 more
doaj   +1 more source

Degradation of two novel congenital TTP ADAMTS13 mutants by the cell proteasome prevents ADAMTS13 secretion [PDF]

, 2016
INTRODUCTION: Over 150 mutations have been identified in the ADAMTS13 gene in patients with congenital thrombotic thrombocytopenic purpura (TTP). The majority of these (86%), lead to reduced (
Garagiola, I, Machin, S, Mackie, I, Peyvandi, F, Underwood, M   +4 more
core   +1 more source