Results 21 to 30 of about 5,761 (161)

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

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

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

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

ADAMTS13 activity and antigen during therapy and follow-up of patients with idiopathic thrombotic thrombocytopenic purpura: correlation with clinical outcome

Haematologica, 2011
Background The assay for ADAMTS13 activity helps clinicians to confirm the clinical diagnosis of idiopathic thrombotic thrombocytopenic purpura. The clinical value of testing for the antigen level of ADAMTS13 protein is, however, less clear.Design and ...
Shangbin Yang, Ming Jin, Shili Lin, Spero Cataland, Haifeng Wu   +4 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

Antibodies against the CUB1-2 domains of ADAMTS13 in a patient with benign monoclonal gammopathy: no causal relationship

Haematologica, 2007
We present a patient with a history of benign monoclonal gammopathy, who developed thrombotic thrombocytopenic purpura (TTP), initially presenting as bilateral serous retinal detachment.
Niels P Riksen, Brenda M Luken, Ina S Klasen, Jan Voorberg, Niels Crama, Marcel van Deuren   +5 more
doaj   +1 more source

The first deletion mutation in the TSP1-6 repeat domain of ADAMTS13 in a family with inherited thrombotic thrombocytopenic purpura

Haematologica, 2009
The inherited deficiency of ADAMTS13 is usually associated with severe forms of thrombotic thrombocytopenic purpura. Among the mutations identified in the ADAMTS13 gene, none have been described on the TSP1-6 repeat domain.
Roberta Palla, Silvia Lavoretano, Rossana Lombardi, Isabella Garagiola, Mehran Karimi, Abdolreza Afrasiabi, Mani Ramzi, Raimondo De Cristofaro, Flora Peyvandi   +8 more
doaj   +1 more source