Results 51 to 60 of about 10,178 (166)

Role of ADAMTS13 in the pathogenesis, diagnosis, and treatment of thrombotic thrombocytopenic purpura

, 2012
The regulation of VWF multimer size is essential in preventing spontaneous microvascular platelet clumping, a central pathophysiologic finding in thrombotic thrombocytopenic purpura (TTP).
Kremer Hovinga, Johanna A, Lämmle, Bernhard   +1 more
core   +1 more source

Open or closed? Understanding the molecular mechanisms and clinical implications of ADAMTS13's conformation

HemaSphere
By proteolyzing prothrombotic von Willebrand factor (VWF) multimers, ADAMTS13 (A Disintegrin And Metalloproteinase with ThromboSpondin type‐1 repeats, member 13) ensures balanced hemostasis and prevents microvascular thrombosis. ADAMTS13's conformational
Quintijn Bonnez, Karen Vanhoorelbeke
doaj   +1 more source

Reduced ADAMTS13 Activity in Correlation with Pathophysiology, Severity, and Outcome of COVID-19: A Retrospective Observational Study

International Journal of Infectious Diseases, 2022
Background: Low ADAMTS13 activity has been suggested to be an interplaying factor in the pathogenesis of COVID-19, considering that it is a thromboinflammatory disease with high risk of microthrombosis.
Wael Hafez, Mohamad Azzam Ziade, Arun Arya, Husam Saleh, Sara Ali, Srinivasa Raghu Rao, Osman Fdl Alla, Mohamed Ali, Mouhamad Al Zouhbi, Ahmed Abdelrahman   +9 more
doaj   +1 more source

Recombinant ADAMTS13 Ameliorates Liver Injury and Improves Hepatic Microcirculation in a Murine Acute‐on‐Chronic Liver Failure Model

Hepatology Research, EarlyView.
ABSTRACT Aim The therapeutic significance of recombinant a disintegrin‐like and metalloprotease with thrombospondin type 1 motif 13 (rADAMTS13) in acute‐on‐chronic liver failure (ACLF) remains unclear. We aimed to investigate the therapeutic effects of rADAMTS13 in a murine model of ACLF‐like liver injury.
Jun‐ichi Hanatani, Norihisa Nishimura, Kosuke Kaji, Masayoshi Takami, Hiroyuki Masuda, Hiroaki Takaya, Koh Kitagawa, Shinya Sato, Tadashi Namisaki, Hitoshi Yoshiji   +9 more
wiley   +1 more source

ADAMTS13 in arterial thrombosis [PDF]

, 2010
The aim of this thesis was to study the role of ADAMTS13 in arterial thrombosis, a disease that comprises both environmental and genetic factors. We determined ADAMTS13 in ischemic stroke patients and controls.
Bongers, T.N. (Tamara)
core   +1 more source

Genetic variants in the ADAMTS13 and SUPT3H genes are associated with ADAMTS13 activity

, 2015
A disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13) cleaves von Willebrand factor, reducing its prothrombotic activity. The genetic determinants of ADAMTS13 activity remain unclear. We performed a genome-wide association study of
Ikram, M.A. (Arfan), Hofman, A. (Albert), Franco, O.H. (Oscar), Boender, J. (Johan), Rottensteiner, H. (Hanspeter), Vries, P.S. (Paul) de, Maat, M.P.M. (Moniek) de, Uitterlinden, A.G. (André), Leebeek, F.W.G. (Frank), Rivadeneira Ramirez, F. (Fernando), Dehghan, A. (Abbas), Sonneveld, M.A.H. (Michelle)   +11 more
core   +2 more sources

Optimization of plasma-based BioID identifies plasminogen as a ligand of ADAMTS13

Scientific Reports
ADAMTS13, a disintegrin and metalloprotease with a thrombospondin type 1 motif, member 13, regulates the length of Von Willebrand factor (VWF) multimers and their platelet-binding activity. ADAMTS13 is constitutively secreted as an active protease and is
Hasam Madarati, Veronica DeYoung, Kanwal Singh, Taylor Sparring, Andrew C. Kwong, James C. Fredenburgh, Cherie Teney, Marlys L. Koschinsky, Michael B. Boffa, Jeffrey I. Weitz, Colin A. Kretz   +10 more
doaj   +1 more source

Generation and validation of small ADAMTS13 fragments for epitope mapping of anti‐ADAMTS13 autoantibodies in immune‐mediated thrombotic thrombocytopenic purpura

Research and Practice in Thrombosis and Haemostasis, 2020
Background In immune‐mediated thrombotic thrombocytopenic purpura (iTTP), patients develop an immune response against the multidomain enzyme ADAMTS13.
Kadri Kangro, Elien Roose, An‐Sofie Schelpe, Edwige Tellier, Gilles Kaplanski, Jan Voorberg, Simon F. De Meyer, Andres Männik, Karen Vanhoorelbeke   +8 more
doaj   +1 more source

Advanced Molecular Analysis in Hemophilia A in a Single Step: Next Generation Sequencing (NGS) and Copy Number Variation (CNV) Analysis

International Journal of Laboratory Hematology, EarlyView.
ABSTRACT Background Hemophilia A, an X‐linked bleeding disorder caused by pathogenic variants in the F8 gene, requires precise genetic diagnosis for optimal management. Conventional stepwise sequence and copy number variation (CNV) analyses are time‐consuming and may leave some cases unresolved.
Enise Avci Durmusalioglu, Esra Isik, Basak Durmus Ozen, Mehmet Mert Topaloglu, Nihal Karadas, Kaan Kavakli, Melike Sezgin Evim, Tolga Aslan, Erşen Kavak, Adalet Meral Gunes, Funda Tayfun Kupesiz, Alphan Kupesiz, Fahri Sahin, Tahir Atik   +13 more
wiley   +1 more source

Longitudinal assessments of plasma ADAMTS13 biomarkers predict recurrence of immune thrombotic thrombocytopenic purpura

Blood Advances, 2019
: Immune thrombotic thrombocytopenic purpura (iTTP) is primarily caused by immunoglobulin G (IgG)–type autoantibodies that bind and inhibit plasma ADAMTS13 activity and/or accelerate its clearance from circulation. Approximately 50% of patients with iTTP
Jingrui Sui, Wenjing Cao, Konstantine Halkidis, Mohammad S. Abdelgawwad, Nicole K. Kocher, Bryan Guillory, Lance A. Williams, Radhika Gangaraju, Marisa B. Marques, X. Long Zheng   +9 more
doaj   +1 more source