ADAMTS-13 gene expression in antiphospholipid syndrome
OBJECTIVE: Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by recurrent thrombosis and fetal mortality. Thrombotic microangiopathy (TMA) is an important histological finding in catastrophic APS (CAPS) and in APS patients with ...
Veysel Sabri Hançer +2 more
doaj +6 more sources
Thrombotic thrombocytopenic purpura with decreased level of ADAMTS-13 activity and increased level of ADAMTS-13 inhibitor in an adolescent [PDF]
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy characterized by endothelial cell damage, resulting in microangiopathic hemolytic anemia, thrombocytopenia, and various degrees of neurological and renal impairment caused by ...
Eun Mi Yang +6 more
doaj +3 more sources
ADAMTS-13 in the Diagnosis and Management of Thrombotic Microangiopathies [PDF]
Thrombotic microangiopathies (TMAs) comprise a group of distinct disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and microvascular thrombosis.
Galit Sarig
doaj +4 more sources
Plasma Levels of ADAMTS-13 Antigen, ADAMTS-13 Inhibitor, ADAMTS-13 Activity, and Von Willebrand Factor in Patients with Behçet's Disease. [PDF]
Behçet's Disease (BD) is characterised by recurrent aphthous oral and genital ulcers. Vascular involvement is one of the poor prognostic factors. Previously, von Willebrand Factor (VWF) has been detected higher in BD compared with healthy controls. We hypothesised that decreased activity or increased inhibitor levels of ADAMTS-13 may cause increased ...
Karasu U +8 more
europepmc +4 more sources
ADAMTS-13 and bleeding phenotype in von Willebrand disease. [PDF]
The bleeding phenotype of von Willebrand disease (VWD) varies highly between patients and can only partly be explained by von Willebrand factor (VWF) parameters. By cleaving large VWF multimers into smaller, less active multimers, ADAMTS-13 is an important regulator of VWF activity.
Boender J +13 more
europepmc +14 more sources
Further characterization of ADAMTS‐13 inactivation by thrombin [PDF]
The multimeric size and platelet-tethering function of von Willebrand factor (VWF) are modulated by the plasma metalloprotease, a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS-13). In vitro ADAMTS-13 is susceptible to proteolytic inactivation by thrombin.In this study, we aimed to characterize the inactivation ...
Lam, JK +4 more
openaire +5 more sources
ADAMTS‐13 glycans and conformation‐dependent activity [PDF]
Essentials The impact of N-linked glycosylation on ADAMTS-13 function has not been fully explored. The activity of glycan modified ADAMTS-13 was investigated under static and shear stress conditions. Terminal sialic acid on the metalloprotease domain glycans are important for ADAMTS-13 activity.
Nowak, AA +6 more
openaire +5 more sources
Therapeutic application of recombinant human ADAMTS-13 improves shock reversal and coagulation status in a trauma hemorrhage and transfusion rat model [PDF]
Introduction In hemorrhaging trauma patients, the endothelium is activated, resulting in excessive endothelial synthesis of von Willebrand Factor (vWF), which may enhance micro-thrombi formation, resulting in obstruction of the microcirculation and ...
Mathijs R. Wirtz +4 more
doaj +2 more sources
Determination of vWF, ADAMTS-13 and Thrombospondin-1 in Venous Thromboembolism and Relating Them to the Presence of Factor V Leiden Mutation [PDF]
Thrombophilia in venous thromboembolism (VTE) is multifactorial. Von Willebrand factor (vWF) plays a major role in primary hemostasis. While elevated vWF levels are well documented in VTE, findings related to its cleaving protease (ADAMTS-13) are ...
Anwar Al-Awadhi PhD +3 more
doaj +2 more sources
Prophylactic use of recombinant ADAMTS-13 during pregnancy for congenital thrombotic thrombocytopenic purpura [PDF]
Background: Congenital thrombotic thrombocytopenic purpura (cTTP) related to ADAMTS-13 deficiency is associated with a maternal risk of death of 10% and a risk of fetal loss greater than 50% without treatment.
Éloïse Colliou +7 more
doaj +2 more sources

