Published in Semin Thromb Hemost on January 01, 1987
von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. N Engl J Med (1998) 6.17
Brief report: variability of thrombosis among homozygous siblings with resistance to activated protein C due to an Arg-->Gln mutation in the gene for factor V. N Engl J Med (1994) 4.00
Deficiency of protein C in congenital thrombotic disease. J Clin Invest (1981) 3.56
A randomized clinical trial of high-intensity warfarin vs. conventional antithrombotic therapy for the prevention of recurrent thrombosis in patients with the antiphospholipid syndrome (WAPS). J Thromb Haemost (2005) 3.47
Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood (1996) 3.39
Protein synthesis by native chemical ligation: expanded scope by using straightforward methodology. Proc Natl Acad Sci U S A (1999) 2.87
Tissue plasminogen activator (tPA) deficiency exacerbates cerebrovascular fibrin deposition and brain injury in a murine stroke model: studies in tPA-deficient mice and wild-type mice on a matched genetic background. Arterioscler Thromb Vasc Biol (1999) 2.56
The incidence of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome: all patients, idiopathic patients, and patients with severe ADAMTS-13 deficiency. J Thromb Haemost (2005) 2.53
Natural history and risk factors for thrombosis in 360 patients with antiphospholipid antibodies: a four-year prospective study from the Italian Registry. Am J Med (1996) 2.51
Accuracy of D-dimer/fibrinogen ratio to predict pulmonary embolism: a prospective diagnostic study. J Thromb Haemost (2003) 2.22
A factor IX-deficient mouse model for hemophilia B gene therapy. Proc Natl Acad Sci U S A (1997) 2.22
Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood (1997) 2.12
Warning: Simplate II--lack of standardization in standardized bleeding time devices. Thromb Haemost (1990) 2.01
Ten years of prophylactic treatment with fresh-frozen plasma in a child with chronic relapsing thrombotic thrombocytopenic purpura as a result of a congenital deficiency of von Willebrand factor-cleaving protease. Br J Haematol (2001) 1.99
Human blood coagulation factor XI. Purification, properties, and mechanism of activation by activated factor XII. J Biol Chem (1977) 1.86
Plasma protein S deficiency in familial thrombotic disease. Blood (1984) 1.82
Human high molecular weight kininogen. Studies of structure-function relationships and of proteolysis of the molecule occurring during contact activation of plasma. J Biol Chem (1979) 1.72
Aetiology and pathogenesis of thrombotic thrombocytopenic purpura and haemolytic uraemic syndrome: the role of von Willebrand factor-cleaving protease. Best Pract Res Clin Haematol (2001) 1.71
American College of Medical Genetics consensus statement on factor V Leiden mutation testing. Genet Med (2001) 1.70
Assay of von Willebrand factor (vWF)-cleaving protease based on decreased collagen binding affinity of degraded vWF: a tool for the diagnosis of thrombotic thrombocytopenic purpura (TTP) Thromb Haemost (1999) 1.69
Anti-inflammatory, antithrombotic, and neuroprotective effects of activated protein C in a murine model of focal ischemic stroke. Circulation (2001) 1.67
Deficiency of protein C inhibitor in combined factor V/VIII deficiency disease. J Clin Invest (1980) 1.61
Mechanisms for the involvement of high molecular weight kininogen in surface-dependent reactions of Hageman factor. Proc Natl Acad Sci U S A (1976) 1.59
Mechanism of action of human activated protein C, a thrombin-dependent anticoagulant enzyme. Blood (1982) 1.58
The binding and cleavage characteristics of human Hageman factor during contact activation. A comparison of normal plasma with plasmas deficient in factor XI, prekallikrein, or high molecular weight kininogen. J Clin Invest (1977) 1.55
Images in clinical medicine. Capnocytophaga canimorsus sepsis. N Engl J Med (1998) 1.55
Association of two silent polymorphisms of platelet glycoprotein Ia/IIa receptor with risk of myocardial infarction: a case-control study. Lancet (1999) 1.54
Measurement of von Willebrand factor-cleaving protease (ADAMTS-13) activity in plasma: a multicenter comparison of different assay methods. J Thromb Haemost (2003) 1.54
Inherited protein C deficiency and coumarin-responsive chronic relapsing purpura fulminans in a newborn infant. Lancet (1983) 1.53
Activation of rabbit Hageman factor by homogenates of cultured rabbit endothelial cells. J Clin Invest (1980) 1.53
Proteolytic inactivation of human factor VIII procoagulant protein by activated human protein C and its analogy with factor V. Blood (1984) 1.52
Role of high-molecular-weight kininogen in surface-binding and activation of coagulation Factor XI and prekallikrein. Proc Natl Acad Sci U S A (1977) 1.52
Inhibition of thrombus formation by activated recombinant protein C in a primate model of arterial thrombosis. Circulation (1990) 1.52
Binding of protein S to factor Va associated with inhibition of prothrombinase that is independent of activated protein C. J Biol Chem (1993) 1.51
Impaired DNase1-mediated degradation of neutrophil extracellular traps is associated with acute thrombotic microangiopathies. J Thromb Haemost (2015) 1.51
Acquired deficiency of von Willebrand factor-cleaving protease in a patient with thrombotic thrombocytopenic purpura. Blood (1998) 1.48
Antithrombotic effects of combining activated protein C and urokinase in nonhuman primates. Circulation (1991) 1.47
Role of surface in surface-dependent activation of Hageman factor (blood coagulation factor XII). Proc Natl Acad Sci U S A (1978) 1.46
Plasma glucosylceramide deficiency as potential risk factor for venous thrombosis and modulator of anticoagulant protein C pathway. Blood (2001) 1.46
Prospective, multicenter validation of prediction scores for major bleeding in elderly patients with venous thromboembolism. J Thromb Haemost (2013) 1.45
von Willebrand factor-cleaving protease in childhood diarrhoea-associated haemolytic uraemic syndrome. Thromb Haemost (2001) 1.43
Effect of hirudin vs heparin on haemostatic activity in patients with acute coronary syndromes; the GUSTO-IIb haemostasis substudy. Eur Heart J (2002) 1.43
Variability of anti-PF4/heparin antibody results obtained by the rapid testing system ID-H/PF4-PaGIA. J Thromb Haemost (2009) 1.41
Prognostic value of protein C concentrations in neutropenic patients at high risk of severe septic complications. Crit Care Med (2000) 1.40
Impairments of the protein C system and fibrinolysis in infection-associated stroke. Stroke (1996) 1.38
Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood (2001) 1.37
The biochemistry and pathophysiology of the contact system of plasma. Adv Immunol (1982) 1.34
Contributions of human platelets to the proteolytic activation of blood coagulation factors XII and XI. Blood (1981) 1.34
ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock. J Thromb Haemost (2007) 1.32
Human factor XII (Hageman factor). Methods Enzymol (1976) 1.31
Protein C, an antithrombotic protein, is reduced in hospitalized patients with intravascular coagulation. Blood (1982) 1.30
Comparison of anticoagulant and procoagulant activities of stimulated platelets and platelet-derived microparticles. Blood (1991) 1.28
Thrombotic events during oral anticoagulant treatment: results of the inception-cohort, prospective, collaborative ISCOAT study: ISCOAT study group (Italian Study on Complications of Oral Anticoagulant Therapy). Thromb Haemost (1997) 1.27
Activated protein C stimulates the fibrinolytic activity of cultured endothelial cells and decreases antiactivator activity. Proc Natl Acad Sci U S A (1985) 1.27
Surface and fluid phase activities of two forms of activated Hageman factor produced during contact activation of plasma. J Exp Med (1978) 1.26
Protein S binds to and inhibits factor Xa. Proc Natl Acad Sci U S A (1994) 1.25
Activated protein C resistance caused by Arg506Gln mutation in factor Va. Lancet (1994) 1.23
Antithrombotic effects of thrombin-induced activation of endogenous protein C in primates. J Clin Invest (1993) 1.17
The frequency of type I heterozygous protein S and protein C deficiency in 141 unrelated young patients with venous thrombosis. Thromb Haemost (1988) 1.17
Direct detection of activated protein C in blood from human subjects. Blood (1992) 1.17
Blood coagulation factor Va abnormality associated with resistance to activated protein C in venous thrombophilia. Blood (1994) 1.16
Reevaluation of the incidence of thromboembolic complications in congenital factor XII deficiency--a study on 73 subjects from 14 Swiss families. Thromb Haemost (1999) 1.16
Alteration of fibrin network by activated protein C. Blood (1994) 1.16
Mechanism of protein C-dependent clot lysis: role of plasminogen activator inhibitor. Blood (1986) 1.15
Protein C supports platelet binding and activation under flow: role of glycoprotein Ib and apolipoprotein E receptor 2. J Thromb Haemost (2008) 1.13
Thrombophilic factors are not the leading cause of thrombosis in Behçet's disease. Ann Rheum Dis (2004) 1.13
Physiologic inhibition of human activated protein C by alpha 1-antitrypsin. J Biol Chem (1988) 1.11
Purification and characterization of plasma protein C inhibitor. Thromb Res (1989) 1.10
Intrinsic stability and functional properties of disulfide bond-stabilized coagulation factor VIIIa variants. J Thromb Haemost (2006) 1.10
Sulfatide-dependent autoactivation of human blood coagulation Factor XII (Hageman Factor). J Biol Chem (1983) 1.09
Clinical studies of protein C. Semin Thromb Hemost (1984) 1.09
Activated protein C resistance: molecular mechanisms based on studies using purified Gln506-factor V. Blood (1995) 1.09
Total chemical synthesis of enzymatically active human type II secretory phospholipase A2. Proc Natl Acad Sci U S A (1997) 1.08
Inhibition of activated protein C by recombinant alpha 1-antitrypsin variants with substitution of arginine or leucine for methionine358. J Biol Chem (1990) 1.08
SimpliRED D-dimer assay: comparability of capillary and citrated venous whole blood, between-assay variability, and performance of the test for exclusion of deep vein thrombosis in symptomatic outpatients. Thromb Haemost (1998) 1.08
Inhibition of platelet-dependent thrombus formation by human activated protein C in a primate model. Blood (1989) 1.08
Human plasma prekallikrein. Studies of its activation by activated factor XII and of its inactivation by diisopropyl phosphofluoridate. Biochemistry (1980) 1.08
Chronic interstitial nephritis in Whipple's disease. Klin Wochenschr (1983) 1.05
A structural model for the prostate disease marker, human prostate-specific antigen. Protein Sci (1994) 1.05
Nonenzymatic anticoagulant activity of the mutant serine protease Ser360Ala-activated protein C mediated by factor Va. Protein Sci (1997) 1.04
Activation of human factor VII in plasma and in purified systems: roles of activated factor IX, kallikrein, and activated factor XII. J Clin Invest (1979) 1.04
Isolation and functional characterization of the active light chain of activated human blood coagulation factor XI. J Biol Chem (1983) 1.04
The effect of phospholipids, calcium ions and protein S on rate constants of human factor Va inactivation by activated human protein C. Eur J Biochem (1992) 1.03
Identification and quantitation of protein S in human platelets. Blood (1985) 1.02
Blood coagulation. The thrombin paradox. Nature (1995) 1.02
An alternative extrinsic pathway of human blood coagulation. Blood (1982) 1.02
Identification of a sequence of human activated protein C (residues 390-404) essential for its anticoagulant activity. J Biol Chem (1991) 1.01
Measurement of ADAMTS-13 activity in plasma by the FRETS-VWF73 assay: comparison with other assay methods. J Thromb Haemost (2006) 1.01
Isolation and characterization of the gene encoding 2,3-oxidosqualene-lanosterol cyclase from Saccharomyces cerevisiae. Proc Natl Acad Sci U S A (1994) 1.00
Receptors for high molecular weight kininogen on stimulated washed human platelets. Biochemistry (1984) 1.00
Human blood coagulation factor IX. Purification, properties, and mechanism of activation by activated factor XI. J Biol Chem (1978) 0.99
Immunochemical studies of human high molecular weight kininogen and of its complexes with plasma prekallikrein or kallikrein. J Biol Chem (1980) 0.99
Deficiency of von Willebrand factor-cleaving protease in familial and acquired thrombotic thrombocytopenic purpura. Baillieres Clin Haematol (1998) 0.99
Activated human protein C prevents thrombin-induced thromboembolism in mice. Evidence that activated protein c reduces intravascular fibrin accumulation through the inhibition of additional thrombin generation. J Clin Invest (1998) 0.99
Binding of coagulation factor XI to washed human platelets. Biochemistry (1986) 0.99
Fibrin glue in surgery: frequent development of inhibitors of bovine thrombin and human factor V. Br J Haematol (1993) 0.98
A novel exosite in the light chain of human activated protein C essential for interaction with blood coagulation factor Va. Biochemistry (1993) 0.98
The autolysis loop of activated protein C interacts with factor Va and differentiates between the Arg506 and Arg306 cleavage sites. Blood (2000) 0.98