Results 21 to 30 of about 5,721 (151)

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

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

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

Phenotypic expression of ADAMTS13 in glomerular endothelial cells.

PLoS ONE, 2011
BackgroundADAMTS13 is the physiological von Willebrand factor (VWF)-cleaving protease. The aim of this study was to examine ADAMTS13 expression in kidneys from ADAMTS13 wild-type (Adamts13⁺/⁺) and deficient (Adamts13⁻/⁻) mice and to investigate the ...
Ramesh Tati, Ann-Charlotte Kristoffersson, Anne-Lie Ståhl, Matthias Mörgelin, David Motto, Simon Satchell, Peter Mathieson, Minola Manea-Hedström, Diana Karpman   +8 more
doaj   +1 more source

Von Willebrand factor, ADAMTS13 and mortality in dialysis patients

BMC Nephrology, 2021
Background Von Willebrand Factor (VWF) multimers are cleaved into smaller and less coagulant forms by the metalloprotease ADAMTS13. The aim of this study was to investigate the association between VWF and ADAMTS13 and mortality in dialysis patients ...
Gurbey Ocak, Mark Roest, Marianne C. Verhaar, Maarten B. Rookmaaker, Peter J. Blankestijn, Willem Jan W. Bos, Rob Fijnheer, Nathalie C. Péquériaux, Friedo W. Dekker   +8 more
doaj   +1 more source

Effect of plasma exchange on COVID-19 associated excess of von Willebrand factor and inflammation in critically ill patients

Scientific Reports, 2022
Ubiquitous microthromboses in the pulmonary vasculature play a crucial role in the pathogenesis of COVID-19 associated acute respiratory distress syndrome (ARDS). Excess of Willebrand factor (vWf) with intravascular multimer formation was identified as a
Felix S. Seibert, Arturo Blazquez-Navarro, Bodo Hölzer, Adrian A. N. Doevelaar, Christian Nusshag, Uta Merle, Christian Morath, Panagiota Zgoura, Rita Dittmer, Sonja Schneppenheim, Jochen Wilhelm, Nina Babel, Ulrich Budde, Timm H. Westhoff   +13 more
doaj   +1 more source

Deficiency of complement factor H-related proteins and autoantibody-positive hemolytic uremic syndrome in an infant with combined partial deficiencies and autoantibodies to complement factor H and ADAMTS13 [PDF]

Clinical Kidney Journal, 2018
A 3-month-old male infant developed an extremely severe episode of atypical hemolytic uremic syndrome (aHUS) associated with partial deficiencies of full-length complement factor H (FH; ∼15% of infant normal) and a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13) (39% of normal) and autoantibodies reactive with
Mini Michael, Nancy Turner, Ewa Elenberg, Linda G Shaffer, Jun Teruya, Mazen Arar, Shiu-Ki Hui, Richard J Smith, Joel Moake   +8 more
openaire   +3 more sources

Von Willebrand Factor and ADAMTS13 in COVID-19 and Beyond: A Question of Balance

European Medical Journal Nephrology, 2021
von Willebrand factor (VWF) is a large, adhesive, multimeric protein involved in haemostasis. The larger the size (or number of VWF multimers), the greater the functionality of the protein. A deficiency or defect in VWF can lead to von Willebrand disease
Emmanuel J Favaloro, Brandon Michael Henry, Giuseppe Lippi   +2 more
doaj  

Three disintegrin-like domain mutations of ADAMTS13： functional deficiency and association with thrombosis [PDF]

Zhenduanxue lilun yu shijian
Objective To analyze the genotypic and phenotypic characteristics of four thrombotic patients carrying heterozygous mutations in the disintegrin-like domain of ADAMTS13 (a disintegrin and metalloproteinase with thrombospondin type 1 motifs 13), and to ...
LIN Liya, WU Xi, MAO Yinqi, CHEN Guangming, WU Wenman, DAI Jing, WANG Xuefeng, DING Qiulan
doaj   +1 more source