Research graph
References from ADAMTS13 as a thromboinflammatory brake at the vascular-immune interface: from viral immunothrombosis to the tumor microenvironment. Local targets link to admitted publications; unresolved targets remain external evidence.
Immune thrombotic thrombocytopenic purpura: a review
10.1001/jama.2025.3807 · 2025 · External reference
ADAMTS13 in the new era of TTP
10.3390/ijms25158137 · 2024 · External reference
Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor
10.1182/blood-2011-02-306597 · 2011 · External reference
Medical consult: aHUS, TTP? How to distinguish and what to do
10.1182/hematology.2023000501 · 2023 · External reference
Thrombosis as an intravascular effector of innate immunity
10.1038/nri3345 · 2013 · External reference
Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor
10.3324/haematol.11677 · 2008 · External reference
The role of ADAMTS-13 in the coagulopathy of sepsis
10.1111/jth.13953 · 2018 · External reference
Effects of convalescent plasma infusion on the ADAMTS13-von Willebrand factor axis and endothelial integrity in patients with severe and critical COVID-19
10.1016/j.rpth.2022.100010 · 2023 · External reference
Distinctive biomarker features in the endotheliopathy of COVID-19 and septic syndromes
10.1097/shk.0000000000001823 · 2022 · External reference
Associations between the von Willebrand factor-ADAMTS13 axis, complement activation, and COVID-19 severity and mortality
10.1055/s-0041-1740182 · 2022 · External reference
Dysregulation of complement components associated with inflammation and coagulation in virally suppressed people living with HIV
10.1093/jimmun/vkaf227 · 2025 · External reference
Hallmarks of cancer: new dimensions
10.1158/2159-8290.cd-21-1059 · 2022 · External reference
Cancer-associated pathways and biomarkers of venous thrombosis
10.1182/blood-2017-03-743211 · 2017 · External reference
Cancer-associated venous thromboembolism
10.1038/s41572-022-00336-y · 2022 · External reference
Cancer-associated thrombosis: enhanced awareness and pathophysiologic complexity
10.1016/j.jtha.2023.02.029 · 2023 · External reference
Immune checkpoint inhibitor-associated thrombotic thrombocytopenic purpura in a patient with metastatic non-small-cell lung cancer
10.7759/cureus.16035 · 2021 · External reference
Case report: thrombotic-thrombocytopenic purpura following ipilimumab and nivolumab combination immunotherapy for metastatic melanoma
10.3389/fimmu.2022.871217 · 2022 · External reference
The role of ADAMTS-13 and von Willebrand factor in cancer patients: results from the Vienna Cancer and Thrombosis Study
10.1002/rth2.12197 · 2019 · External reference
Prognostic value of von Willebrand factor levels in patients with metastatic melanoma treated by immune checkpoint inhibitors
10.1136/jitc-2022-006456 · 2023 · External reference
ADAMTS13 protease or lack of von Willebrand factor protects irradiation and melanoma-induced thrombotic microangiopathy in zebrafish
10.1111/jth.15820 · 2022 · External reference
von Willebrand factor fibers promote cancer-associated platelet aggregation in Malignant melanoma of mice and humans
10.1182/blood-2014-08-595686 · 2015 · External reference
Functional architecture of Weibel-Palade bodies
10.1182/blood-2010-09-267492 · 2011 · External reference
Functional self-association of von Willebrand factor during platelet adhesion under flow
10.1073/pnas.012459599 · 2002 · External reference
Mechanism of von Willebrand factor scissile bond cleavage by a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)
10.1073/pnas.1018559108 · 2011 · External reference
Crystal structure and substrate-induced activation of ADAMTS13
10.1038/s41467-019-11474-5 · 2019 · External reference
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor
10.1073/pnas.0909755106 · 2009 · External reference
An integrative structural biology analysis of von Willebrand factor binding and processing by ADAMTS-13 in solution
10.1016/j.jmb.2021.166954 · 2021 · External reference
Allosteric activation of ADAMTS13 by von Willebrand factor
10.1073/pnas.1413282112 · 2014 · External reference
The contribution of the sinusoidal endothelial cell receptors CLEC4M, stabilin-2, and SCARA5 to VWF-FVIII clearance in thrombosis and hemostasis
10.1016/j.jtha.2023.04.014 · 2023 · External reference
The endothelial lectin clearance receptor CLEC4M binds and internalizes factor VIII in a VWF-dependent and independent manner
10.1111/jth.14404 · 2019 · External reference
Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo
10.1182/blood-2008-01-133181 · 2008 · External reference
Siglec-5 and Siglec-14 mediate the endocytosis of ADAMTS13
10.1016/j.thromres.2022.09.005 · 2022 · External reference
Clearance of VWF by hepatic macrophages is critical for the protective effect of ADAMTS13 in sickle cell anemia mice
10.1182/blood.2023021583 · 2024 · External reference
International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13
10.1111/ijlh.13295 · 2020 · External reference
ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease
10.1182/bloodadvances.2020003265 · 2021 · External reference
Inverse regulation of confluence-dependent ADAMTS13 and von Willebrand factor expression in human endothelial cells
10.1055/s-0041-1733800 · 2022 · External reference
Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow
10.1182/blood-2004-01-0107 · 2004 · External reference
Hypochlorous acid generated by neutrophils inactivates ADAMTS13: an oxidative mechanism for regulating ADAMTS13 proteolytic activity during inflammation
10.1074/jbc.m114.599084 · 2015 · External reference
Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13
10.1182/blood-2009-03-213967 · 2010 · External reference
SARS-CoV-2 S protein activates NLRP3 inflammasome and deregulates coagulation factors in endothelial and immune cells
10.1186/s12964-023-01397-6 · 2024 · External reference
A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear
10.1016/j.jtha.2024.11.031 · 2025 · External reference
Anti-C5a antibody vilobelimab treatment and the effect on biomarkers of inflammation and coagulation in patients with severe COVID-19: a substudy of the phase 2 PANAMO trial
10.1186/s12931-022-02278-1 · 2022 · External reference
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis
10.1111/jth.14686 · 2020 · External reference
Extracellular DNA traps promote thrombosis
10.1073/pnas.1005743107 · 2010 · External reference
Neutrophil extracellular traps promote deep vein thrombosis in mice
10.1111/j.1538-7836.2011.04544.x · 2012 · External reference
von Willebrand factor is a cofactor in complement regulation
10.1182/blood-2014-06-585430 · 2015 · External reference
Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis
10.1371/journal.pone.0059372 · 2013 · External reference
Complement activation associated with ADAMTS13 deficiency in human and murine thrombotic microangiopathy
10.4049/jimmunol.1301221 · 2013 · External reference
Modulation of the antitumor immune response by complement
10.1038/ni.1655 · 2008 · External reference
Recombinant ADAMTS13 in congenital thrombotic thrombocytopenic purpura
10.1056/nejmoa2314793 · 2024 · External reference
Apadamtase alfa: First approval
10.1007/s40265-024-02007-6 · 2024 · External reference
Successful treatment of acquired thrombotic thrombocytopenic purpura with caplacizumab combined with plasma exchanges and immune suppression in 3 children
10.1097/mph.0000000000002843 · 2024 · External reference
Von Willebrand factor, endothelial dysfunction, and cardiovascular disease
10.1111/j.1538-7836.2006.01949.x · 2006 · External reference
Tumor-derived matrix metalloproteinase-1 targets endothelial proteinase-activated receptor 1 promoting endothelial cell activation
10.1158/0008-5472.can-05-3897 · 2006 · External reference
Inflammation and the blood microvascular system
10.1101/cshperspect.a016345 · 2014 · External reference
Evolving functions of endothelial cells in inflammation
10.1038/nri2171 · 2007 · External reference
Von Willebrand factor targets IL-8 to Weibel-Palade bodies in an endothelial cell line
10.1016/s0014-4827(03)00094-6 · 2003 · External reference
Chemotherapeutic agents doxorubicin and epirubicin induce a procoagulant phenotype on endothelial cells and blood monocytes
10.1111/j.1538-7836.2009.03300.x · 2009 · External reference
Doxorubicin-induced vascular toxicity--targeting potential pathways may reduce procoagulant activity
10.1371/journal.pone.0075157 · 2013 · External reference
Comparative endothelial profiling of doxorubicin and daunorubicin in cultured endothelial cells
10.1016/j.tiv.2014.12.009 · 2015 · External reference
Radiation-induced von Willebrand factor release
10.1182/blood.v90.5.2109 · 1997 · External reference
In vitro and in vivo expression of endothelial von Willebrand factor and leukocyte accumulation after fractionated irradiation
10.1667/0033-7587(2000)154[0375:ivaive]2.0.co;2 · 2000 · External reference
Vascular damage as an underlying mechanism of cardiac and cerebral toxicity in irradiated cancer patients
10.1667/rr1862.1 · 2010 · External reference
Association between immune checkpoint inhibitors with cardiovascular events and atherosclerotic plaque
10.1161/circulationaha.120.049981 · 2020 · External reference
Complement in cancer: Untangling an intricate relationship
10.1038/nri.2017.97 · 2018 · External reference
Tissue factor-expressing tumor-derived extracellular vesicles activate quiescent endothelial cells via protease-activated receptor-1
10.3389/fonc.2017.00261 · 2017 · External reference
Tissue factor and extracellular vesicles: Activation of coagulation and impact on survival in cancer
10.3390/cancers13153839 · 2021 · External reference
Contributing role of extracellular vesicles on vascular endothelium haemostatic balance in cancer
10.3402/jev.v3.24400 · 2014 · External reference
An expendable player in positive vascular remodeling? ADAMTS13 deficiency does not affect arteriogenesis or angiogenesis
10.3390/ijms26189137 · 2025 · External reference
Antiangiogenesis strategies revisited: From starving tumors to alleviating hypoxia
10.1016/j.ccell.2014.10.006 · 2014 · External reference
Immune-mediated TTP secondary to checkpoint inhibitor use in a patient with stage IV melanoma
10.1136/bcr-2024-263705 · 2025 · External reference
Presumed complement-mediated, checkpoint inhibitor-induced, thrombotic microangiopathy in a patient with metastatic melanoma
10.1136/bcr-2021-242075 · 2021 · External reference
Incidence of thromboembolism in patients with melanoma on immune checkpoint inhibitor therapy and its adverse association with survival
10.1136/jitc-2020-001719 · 2021 · External reference
Chemotherapy-associated thrombotic microangiopathy
10.34067/kid.0000000000000061 · 2023 · External reference
A case of gemcitabine-induced thrombotic microangiopathy treated with ravulizumab in a patient with stage IV pancreatic cancer
10.7759/cureus.13031 · 2021 · External reference
Unresolved reference
External reference
Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura
10.1056/nejmoa1806311 · 2019 · External reference
Immune thrombotic thrombocytopenic purpura: a review
10.1001/jama.2025.3807 · ExternalCitation · doi-reference
The role of ADAMTS-13 and von Willebrand factor in cancer patients: results from the Vienna Cancer and Thrombosis Study
10.1002/rth2.12197 · ExternalCitation · doi-reference
Apadamtase alfa: First approval
10.1007/s40265-024-02007-6 · ExternalCitation · doi-reference
Antiangiogenesis strategies revisited: From starving tumors to alleviating hypoxia
10.1016/j.ccell.2014.10.006 · ExternalCitation · doi-reference
An integrative structural biology analysis of von Willebrand factor binding and processing by ADAMTS-13 in solution
10.1016/j.jmb.2021.166954 · ExternalCitation · doi-reference
Cancer-associated thrombosis: enhanced awareness and pathophysiologic complexity
10.1016/j.jtha.2023.02.029 · ExternalCitation · doi-reference
The contribution of the sinusoidal endothelial cell receptors CLEC4M, stabilin-2, and SCARA5 to VWF-FVIII clearance in thrombosis and hemostasis
10.1016/j.jtha.2023.04.014 · ExternalCitation · doi-reference
A mutant complement factor H (W1183R) enhances proteolytic cleavage of von Willebrand factor by ADAMTS-13 under shear
10.1016/j.jtha.2024.11.031 · ExternalCitation · doi-reference
Effects of convalescent plasma infusion on the ADAMTS13-von Willebrand factor axis and endothelial integrity in patients with severe and critical COVID-19
10.1016/j.rpth.2022.100010 · ExternalCitation · doi-reference
Siglec-5 and Siglec-14 mediate the endocytosis of ADAMTS13
10.1016/j.thromres.2022.09.005 · ExternalCitation · doi-reference
Comparative endothelial profiling of doxorubicin and daunorubicin in cultured endothelial cells
10.1016/j.tiv.2014.12.009 · ExternalCitation · doi-reference
Von Willebrand factor targets IL-8 to Weibel-Palade bodies in an endothelial cell line
10.1016/s0014-4827(03)00094-6 · ExternalCitation · doi-reference
Modulation of the antitumor immune response by complement
10.1038/ni.1655 · ExternalCitation · doi-reference
Complement in cancer: Untangling an intricate relationship
10.1038/nri.2017.97 · ExternalCitation · doi-reference
Evolving functions of endothelial cells in inflammation
10.1038/nri2171 · ExternalCitation · doi-reference
Thrombosis as an intravascular effector of innate immunity
10.1038/nri3345 · ExternalCitation · doi-reference
Crystal structure and substrate-induced activation of ADAMTS13
10.1038/s41467-019-11474-5 · ExternalCitation · doi-reference
Cancer-associated venous thromboembolism
10.1038/s41572-022-00336-y · ExternalCitation · doi-reference
Inverse regulation of confluence-dependent ADAMTS13 and von Willebrand factor expression in human endothelial cells
10.1055/s-0041-1733800 · ExternalCitation · doi-reference
Associations between the von Willebrand factor-ADAMTS13 axis, complement activation, and COVID-19 severity and mortality
10.1055/s-0041-1740182 · ExternalCitation · doi-reference
Caplacizumab treatment for acquired thrombotic thrombocytopenic purpura
10.1056/nejmoa1806311 · ExternalCitation · doi-reference
Recombinant ADAMTS13 in congenital thrombotic thrombocytopenic purpura
10.1056/nejmoa2314793 · ExternalCitation · doi-reference
Functional self-association of von Willebrand factor during platelet adhesion under flow
10.1073/pnas.012459599 · ExternalCitation · doi-reference
Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor
10.1073/pnas.0909755106 · ExternalCitation · doi-reference
Extracellular DNA traps promote thrombosis
10.1073/pnas.1005743107 · ExternalCitation · doi-reference
Mechanism of von Willebrand factor scissile bond cleavage by a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13)
10.1073/pnas.1018559108 · ExternalCitation · doi-reference
Allosteric activation of ADAMTS13 by von Willebrand factor
10.1073/pnas.1413282112 · ExternalCitation · doi-reference
Hypochlorous acid generated by neutrophils inactivates ADAMTS13: an oxidative mechanism for regulating ADAMTS13 proteolytic activity during inflammation
10.1074/jbc.m114.599084 · ExternalCitation · doi-reference
Dysregulation of complement components associated with inflammation and coagulation in virally suppressed people living with HIV
10.1093/jimmun/vkaf227 · ExternalCitation · doi-reference
Successful treatment of acquired thrombotic thrombocytopenic purpura with caplacizumab combined with plasma exchanges and immune suppression in 3 children
10.1097/mph.0000000000002843 · ExternalCitation · doi-reference
Distinctive biomarker features in the endotheliopathy of COVID-19 and septic syndromes
10.1097/shk.0000000000001823 · ExternalCitation · doi-reference
Inflammation and the blood microvascular system
10.1101/cshperspect.a016345 · ExternalCitation · doi-reference
International Council for Standardization in Haematology (ICSH) recommendations for laboratory measurement of ADAMTS13
10.1111/ijlh.13295 · ExternalCitation · doi-reference
Von Willebrand factor, endothelial dysfunction, and cardiovascular disease
10.1111/j.1538-7836.2006.01949.x · ExternalCitation · doi-reference
Chemotherapeutic agents doxorubicin and epirubicin induce a procoagulant phenotype on endothelial cells and blood monocytes
10.1111/j.1538-7836.2009.03300.x · ExternalCitation · doi-reference
Neutrophil extracellular traps promote deep vein thrombosis in mice
10.1111/j.1538-7836.2011.04544.x · ExternalCitation · doi-reference
The role of ADAMTS-13 in the coagulopathy of sepsis
10.1111/jth.13953 · ExternalCitation · doi-reference
The endothelial lectin clearance receptor CLEC4M binds and internalizes factor VIII in a VWF-dependent and independent manner
10.1111/jth.14404 · ExternalCitation · doi-reference
Von Willebrand factor and ADAMTS13 impact on the outcome of Staphylococcus aureus sepsis
10.1111/jth.14686 · ExternalCitation · doi-reference
ADAMTS13 protease or lack of von Willebrand factor protects irradiation and melanoma-induced thrombotic microangiopathy in zebrafish
10.1111/jth.15820 · ExternalCitation · doi-reference
Presumed complement-mediated, checkpoint inhibitor-induced, thrombotic microangiopathy in a patient with metastatic melanoma
10.1136/bcr-2021-242075 · ExternalCitation · doi-reference
Immune-mediated TTP secondary to checkpoint inhibitor use in a patient with stage IV melanoma
10.1136/bcr-2024-263705 · ExternalCitation · doi-reference
Incidence of thromboembolism in patients with melanoma on immune checkpoint inhibitor therapy and its adverse association with survival
10.1136/jitc-2020-001719 · ExternalCitation · doi-reference
Prognostic value of von Willebrand factor levels in patients with metastatic melanoma treated by immune checkpoint inhibitors
10.1136/jitc-2022-006456 · ExternalCitation · doi-reference
Tumor-derived matrix metalloproteinase-1 targets endothelial proteinase-activated receptor 1 promoting endothelial cell activation
10.1158/0008-5472.can-05-3897 · ExternalCitation · doi-reference
Hallmarks of cancer: new dimensions
10.1158/2159-8290.cd-21-1059 · ExternalCitation · doi-reference
Association between immune checkpoint inhibitors with cardiovascular events and atherosclerotic plaque
10.1161/circulationaha.120.049981 · ExternalCitation · doi-reference
Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow
10.1182/blood-2004-01-0107 · ExternalCitation · doi-reference
Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo
10.1182/blood-2008-01-133181 · ExternalCitation · doi-reference
Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13
10.1182/blood-2009-03-213967 · ExternalCitation · doi-reference
Functional architecture of Weibel-Palade bodies
10.1182/blood-2010-09-267492 · ExternalCitation · doi-reference
Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor
10.1182/blood-2011-02-306597 · ExternalCitation · doi-reference
von Willebrand factor is a cofactor in complement regulation
10.1182/blood-2014-06-585430 · ExternalCitation · doi-reference
von Willebrand factor fibers promote cancer-associated platelet aggregation in Malignant melanoma of mice and humans
10.1182/blood-2014-08-595686 · ExternalCitation · doi-reference
Cancer-associated pathways and biomarkers of venous thrombosis
10.1182/blood-2017-03-743211 · ExternalCitation · doi-reference
Clearance of VWF by hepatic macrophages is critical for the protective effect of ADAMTS13 in sickle cell anemia mice
10.1182/blood.2023021583 · ExternalCitation · doi-reference
Radiation-induced von Willebrand factor release
10.1182/blood.v90.5.2109 · ExternalCitation · doi-reference
ASH ISTH NHF WFH 2021 guidelines on the diagnosis of von Willebrand disease
10.1182/bloodadvances.2020003265 · ExternalCitation · doi-reference
Medical consult: aHUS, TTP? How to distinguish and what to do
10.1182/hematology.2023000501 · ExternalCitation · doi-reference
Anti-C5a antibody vilobelimab treatment and the effect on biomarkers of inflammation and coagulation in patients with severe COVID-19: a substudy of the phase 2 PANAMO trial
10.1186/s12931-022-02278-1 · ExternalCitation · doi-reference
SARS-CoV-2 S protein activates NLRP3 inflammasome and deregulates coagulation factors in endothelial and immune cells
10.1186/s12964-023-01397-6 · ExternalCitation · doi-reference
Assembly and activation of alternative complement components on endothelial cell-anchored ultra-large von Willebrand factor links complement and hemostasis-thrombosis
10.1371/journal.pone.0059372 · ExternalCitation · doi-reference
Doxorubicin-induced vascular toxicity--targeting potential pathways may reduce procoagulant activity
10.1371/journal.pone.0075157 · ExternalCitation · doi-reference
In vitro and in vivo expression of endothelial von Willebrand factor and leukocyte accumulation after fractionated irradiation
10.1667/0033-7587(2000)154[0375:ivaive]2.0.co;2 · ExternalCitation · doi-reference
Vascular damage as an underlying mechanism of cardiac and cerebral toxicity in irradiated cancer patients
10.1667/rr1862.1 · ExternalCitation · doi-reference
Inflammation-associated ADAMTS13 deficiency promotes formation of ultra-large von Willebrand factor
10.3324/haematol.11677 · ExternalCitation · doi-reference
Case report: thrombotic-thrombocytopenic purpura following ipilimumab and nivolumab combination immunotherapy for metastatic melanoma
10.3389/fimmu.2022.871217 · ExternalCitation · doi-reference
Tissue factor-expressing tumor-derived extracellular vesicles activate quiescent endothelial cells via protease-activated receptor-1
10.3389/fonc.2017.00261 · ExternalCitation · doi-reference
Tissue factor and extracellular vesicles: Activation of coagulation and impact on survival in cancer
10.3390/cancers13153839 · ExternalCitation · doi-reference
ADAMTS13 in the new era of TTP
10.3390/ijms25158137 · ExternalCitation · doi-reference
An expendable player in positive vascular remodeling? ADAMTS13 deficiency does not affect arteriogenesis or angiogenesis
10.3390/ijms26189137 · ExternalCitation · doi-reference
Contributing role of extracellular vesicles on vascular endothelium haemostatic balance in cancer
10.3402/jev.v3.24400 · ExternalCitation · doi-reference
Chemotherapy-associated thrombotic microangiopathy
10.34067/kid.0000000000000061 · ExternalCitation · doi-reference
Complement activation associated with ADAMTS13 deficiency in human and murine thrombotic microangiopathy
10.4049/jimmunol.1301221 · ExternalCitation · doi-reference
A case of gemcitabine-induced thrombotic microangiopathy treated with ravulizumab in a patient with stage IV pancreatic cancer
10.7759/cureus.13031 · ExternalCitation · doi-reference
Immune checkpoint inhibitor-associated thrombotic thrombocytopenic purpura in a patient with metastatic non-small-cell lung cancer
10.7759/cureus.16035 · ExternalCitation · doi-reference
