Synonymous ADAMTS13 variants impact molecular characteristics and contribute to variability in active protein abundance [PDF]
Blood Advances, 2022 The effects of synonymous single nucleotide variants (sSNVs) are often neglected since they do not alter protein primary structure. Nevertheless, there is growing evidence that synonymous variations may impact mRNA expression, protein conformation and activity which may lead to protein deficiency and disease manifestations.
Katarzyna Izabela Jankowska +8 more
semanticscholar +4 more sources
Decreased protein C activity, lower ADAMTS13 antigen and free protein S levels accompanied by unchanged thrombin generation potential in hospitalized COVID-19 patients [PDF]
Thrombosis Research, 2023 COVID-19 is associated with an increased thromboembolic risk. However, the mechanisms triggering clot formation in those patients remain unknown.In 118 adult Caucasian severe but non-critically ill COVID-19 patients (median age 58 years; 73 % men) and 46 controls, we analyzed in vitro plasma thrombin generation profile (calibrated automated thrombogram
Wójcik, Krzysztof +13 more
semanticscholar +5 more sources
Improvement of recombinant ADAMTS13 production through a more optimal signal peptide or an N‐terminal fusion protein [PDF]
Journal of Thrombosis and Haemostasis, 2022 Recombinant human ADAMTS13 (rADAMTS13) is a key protein in fundamental research for investigating its mode of action and the pathophysiology of thrombotic thrombocytopenic purpura (TTP). However, the expression of rADAMTS13 is quite low in mammalian cells, which makes the production of the protein time-consuming and labor-intensive.We aimed at ...
Kadri Kangro +8 more
semanticscholar +4 more sources
Local Elongation of Endothelial Cell-anchored von Willebrand Factor Strings Precedes ADAMTS13 Protein-mediated Proteolysis [PDF]
Journal of Biological Chemistry, 2011 Platelet-decorated von Willebrand factor (VWF) strings anchored to the endothelial surface are rapidly cleaved by ADAMTS13. Individual VWF string characteristics such as number, location, and auxiliary features of the ADAMTS13 cleavage sites were explored here using imaging and computing software.
Hiroshi Uji-i +7 more
semanticscholar +6 more sources
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.
John Cannon +14 more
semanticscholar +3 more sources
Thirty five novel nsSNPs may effect onADAMTS13protein leading to Thrombotic thrombocytopenic purpura (TTP) using bioinformatics approach [PDF]
bioRxiv, 2019 ABSTRACTBackgroundGenetic polymorphisms in theADAMTS13gene are associated with thrombotic thrombocytopenic purpura or TTP, a life-threatening microangiopathic disorder. This study aims to predict the possible pathogenic SNPs of this gene and their impact on the protein structure and function using insilico methods.MethodsSNPs retrieved from the NCBI ...
Abdelhameed, Tebyan A. +12 more
semanticscholar +4 more sources
ADAMTS13, an Anti-thrombotic Protein: Evidence Outside of Thrombotic Thrombocytopenic Purpura
Blood, 2019 von Willebrand factor (VWF) is a large multimeric plasma glycoprotein that plays a crucial role in hemostasis and thrombosis. VWF recruits platelets at sites of vascular injury by acting as a molecular bridge between circulating platelets and the site of injured or activated blood vessels.
Simon F. De Meyer
semanticscholar +4 more sources
Preserved Expression of mRNA Coding von Willebrand Factor-Cleaving Protease ADAMTS13 by Selenite and Activated Protein C [PDF]
Molecular Medicine, 2015 In sepsis, the severity-dependent decrease of von Willebrand factor (VWF)-inactivating protease, a disintegrin and metalloproteinase with thrombospondin motifs 13 (ADAMTS13), results in platelet aggregation and consumption, leading to sepsis-associated thrombotic microangiopathy (TMA) and organ failure.
Michael Liembo Ekaney +10 more
semanticscholar +5 more sources
Overview of ADAMTS13 Protein in Diagnosis and Patient Management of TTP
Documenta Haematologica - Revista Romana de HematologieThe accurate diagnosis and effective management of Thrombotic Thrombocytopenic Purpura (TTP), a rare but life-threatening hematologic disorder, rely critically on the assessment of ADAMTS13 activity. ADAMTS13, a von Willebrand factor-cleaving protease, plays a pivotal role in the pathophysiology of TTP. This review synthesizes current understanding and
Georgiana GHERGHE +7 more
semanticscholar +3 more sources
Anti-ADAMTS13 Autoantibodies in Immune-Mediated Thrombotic Thrombocytopenic Purpura [PDF]
AntibodiesAutoantibodies to ADAMTS13 are at the center of pathology of the immune-mediated thrombotic thrombocytopenic purpura. These autoantibodies can be either inhibitory (enzymatic function) or non-inhibitory, resulting in protein depletion.
Michael R. Snyder, Robert W. Maitta
doaj +3 more sources