Published in J Clin Invest on November 01, 1980
Deficiency of protein C in congenital thrombotic disease. J Clin Invest (1981) 3.56
Evidence of normal functional levels of activated protein C inhibitor in combined Factor V/VIII deficiency disease. J Clin Invest (1982) 1.60
Glycosaminoglycans and the regulation of blood coagulation. Biochem J (1993) 1.57
Generation of fibrinolytic activity by infusion of activated protein C into dogs. J Clin Invest (1981) 1.50
Activation of protein C in vivo. J Clin Invest (1982) 1.20
Evolution and organization of the human protein C gene. Proc Natl Acad Sci U S A (1986) 1.14
Disruption of the protein C inhibitor gene results in impaired spermatogenesis and male infertility. J Clin Invest (2000) 1.13
Protein C inhibitor in human body fluids. Seminal plasma is rich in inhibitor antigen deriving from cells throughout the male reproductive system. J Clin Invest (1992) 1.09
The locus for combined factor V-factor VIII deficiency (F5F8D) maps to 18q21, between D18S849 and D18S1103. Am J Hum Genet (1997) 1.04
The COPII pathway and hematologic disease. Blood (2012) 1.03
Detection of a new heparin-dependent inhibitor of thrombin in human plasma. J Clin Invest (1981) 1.00
Human coagulation factor Va is a cofactor for the activation of protein C. Proc Natl Acad Sci U S A (1983) 0.99
Molecular basis of thrombin recognition by protein C inhibitor revealed by the 1.6-A structure of the heparin-bridged complex. Proc Natl Acad Sci U S A (2008) 0.98
Elucidating the structural chemistry of glycosaminoglycan recognition by protein C inhibitor. Proc Natl Acad Sci U S A (1990) 0.96
Linkage of combined factors V and VIII deficiency to chromosome 18q by homozygosity mapping. J Clin Invest (1997) 0.94
Protein C inhibitor is expressed in tubular cells of human kidney. J Clin Invest (1994) 0.86
Anticoagulant synergism of heparin and activated protein C in vitro. Role of a novel anticoagulant mechanism of heparin, enhancement of inactivation of factor V by activated protein C. J Clin Invest (1997) 0.83
Heterogeneity of human factor V deficiency. Evidence for the existence of antigen-positive variants. J Clin Invest (1983) 0.79
Interaction of protein C inhibitor with the type II transmembrane serine protease enteropeptidase. PLoS One (2012) 0.78
TFPI1 mediates resistance to doxorubicin in breast cancer cells by inducing a hypoxic-like response. PLoS One (2014) 0.77
Continuing education course #2: current understanding of hemostasis. Toxicol Pathol (2010) 0.76
Protein C inhibitor (PCI) binds to phosphatidylserine exposing cells with implications in the phagocytosis of apoptotic cells and activated platelets. PLoS One (2014) 0.76
A+-helix of protein C inhibitor (PCI) is a cell-penetrating peptide that mediates cell membrane permeation of PCI. J Biol Chem (2014) 0.76
Combined factors V and VIII deficiency climbs onto the map. J Clin Invest (1997) 0.75
Phospholipid Binding Protein C Inhibitor (PCI) Is Present on Microparticles Generated In Vitro and In Vivo. PLoS One (2015) 0.75
Inhibition of activated protein C by platelets. J Clin Invest (1989) 0.75
Is protein C inhibitor antithrombotic and protective in pulmonary hypertension? J Thromb Haemost (2006) 0.75
Expression patterns of protein C inhibitor in mouse development. J Mol Histol (2010) 0.75
A new vitamin K-dependent protein. Purification from bovine plasma and preliminary characterization. J Biol Chem (1976) 3.57
Anticoagulant properties of bovine plasma protein C following activation by thrombin. Biochemistry (1977) 2.96
Preparation and properties of bovine factor VIII (antihemophilic factor). Biochemistry (1980) 2.89
Human plasma protein C: isolation, characterization, and mechanism of activation by alpha-thrombin. J Clin Invest (1979) 2.18
Proteolytic activation of protein C from bovine plasma. Biochemistry (1976) 2.12
A new vitamin K-dependent protein. A phospholipid-binding zymogen of a serine esterase. J Biol Chem (1976) 1.64
Activated protein C inhibits platelet prothrombin-converting activity. Blood (1979) 1.51
[Congenital factor V deficiency (parahemophilia) with true hemophilia in two brothers]. Bibl Paediatr (1954) 1.48
Inhibitory effect of activated protein C on activation of prothrombin by platelet-bound factor Xa. Eur J Biochem (1980) 1.31
Human factor XII (Hageman factor). Methods Enzymol (1976) 1.31
Human plasma prekallikrein. Studies of its activation by activated factor XII and of its inactivation by diisopropyl phosphofluoridate. Biochemistry (1980) 1.08
Immunological studies in combined factor V and factor VIII deficiency. Br J Haematol (1977) 1.00
Inhibitor of human blood coagulation elicited by thrombin. J Lab Clin Med (1972) 0.94
Congenital familial deficiency of factor V (parahaemophilia) combined with deficiency of antihaemophilic globulin. Br J Haematol (1956) 0.94
Relationship of "new" vitamin K-dependent Protein C and "old" autoprothrombin II-a. Thromb Res (1976) 0.94
The mechanism of activation of bovine prothrombin by an activator isolated from Echis carinatus venon and characterization of the new active intermediates. J Biochem (1976) 0.92
Combined factor-V and factor-VIII deficiency: report of four cases. Br J Haematol (1969) 0.88
Combined deficiency of factor V and factor VIII: report of a family and genetic analysis. Br J Haematol (1972) 0.83
Inherited combined deficiency of factor V and factor VIII: report of a case with normal factor VIII antigen and ristocetin-induced platelet aggregation. Am J Hematol (1977) 0.82
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
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
A factor IX-deficient mouse model for hemophilia B gene therapy. Proc Natl Acad Sci U S A (1997) 2.22
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
American College of Medical Genetics consensus statement on factor V Leiden mutation testing. Genet Med (2001) 1.70
Anti-inflammatory, antithrombotic, and neuroprotective effects of activated protein C in a murine model of focal ischemic stroke. Circulation (2001) 1.67
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
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
Inhibition of thrombus formation by activated recombinant protein C in a primate model of arterial thrombosis. Circulation (1990) 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
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
Antithrombotic effects of combining activated protein C and urokinase in nonhuman primates. Circulation (1991) 1.47
Plasma glucosylceramide deficiency as potential risk factor for venous thrombosis and modulator of anticoagulant protein C pathway. Blood (2001) 1.46
Role of surface in surface-dependent activation of Hageman factor (blood coagulation factor XII). Proc Natl Acad Sci U S A (1978) 1.46
A 5.3-kb deletion including exon XIII of the protein S alpha gene occurs in two protein S-deficient families. Blood (1991) 1.45
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
Prognostic value of protein C concentrations in neutropenic patients at high risk of severe septic complications. Crit Care Med (2000) 1.40
Activated partial thromboplastin time reagent sensitivity to the presence of the lupus anticoagulant. Arch Pathol Lab Med (1992) 1.38
Impairments of the protein C system and fibrinolysis in infection-associated stroke. Stroke (1996) 1.38
Severe protein C deficiency in newborn infants. J Pediatr (1988) 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
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
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
Severe homozygous protein C deficiency. J Pediatr (1984) 1.20
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
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
Coagulation and fibrinolytic profiles in patients with severe pulmonary hypertension. Chest (1996) 1.14
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
Human endothelial cells synthesize protein S. Blood (1986) 1.12
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
High-affinity RNA ligands to human alpha-thrombin. Nucleic Acids Res (1994) 1.10
Sulfatide-dependent autoactivation of human blood coagulation Factor XII (Hageman Factor). J Biol Chem (1983) 1.09
Neonatal purpura fulminans associated with homozygous protein S deficiency. Lancet (1990) 1.09
Activated protein C resistance: molecular mechanisms based on studies using purified Gln506-factor V. Blood (1995) 1.09
Biosynthesis and secretion of factor VII, protein C, protein S, and the Protein C inhibitor from a human hepatoma cell line. Blood (1986) 1.09
Clinical studies of protein C. Semin Thromb Hemost (1984) 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
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
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
Isolation and functional characterization of the active light chain of activated human blood coagulation factor XI. J Biol Chem (1983) 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
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
Blood coagulation. The thrombin paradox. Nature (1995) 1.02
Identification and quantitation of protein S in human platelets. Blood (1985) 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
Neonatal purpura fulminans: a genetic disorder related to the absence of protein C in blood. Blood (1985) 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
Proposed classification and pathologic mechanisms of purpura fulminans and skin necrosis. Semin Thromb Hemost (1990) 0.99
Binding of coagulation factor XI to washed human platelets. Biochemistry (1986) 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
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
Rabbit blood coagulation factor XI. Purification and properties. Thromb Res (1979) 0.97
Immunoblotting studies of the molecular forms of protein C in plasma. Thromb Res (1988) 0.97
A protein S deficient family with portal vein thrombosis. Thromb Haemost (1985) 0.96
Autoactivation of human plasma prekallikrein. J Biol Chem (1987) 0.96
Elucidating the structural chemistry of glycosaminoglycan recognition by protein C inhibitor. Proc Natl Acad Sci U S A (1990) 0.96
Conformational changes in aspartate trancarbamylase. I. Studies of ligand binding and of subunit interactions by circular dichroism spectroscopy. J Biol Chem (1972) 0.95
Cardiolipin is a normal component of human plasma lipoproteins. Proc Natl Acad Sci U S A (2000) 0.95
Immunological studies of prekallikrein, kallikrein, and high-molecular-weight kininogen in normal and deficient plasmas and in normal plasma after cold-dependent activation. J Lab Clin Med (1980) 0.94
Human protein C: inactivation of factors V and VIII in plasma by the activated molecule. Ann N Y Acad Sci (1981) 0.94
Disulfide bond-stabilized factor VIII has prolonged factor VIIIa activity and improved potency in whole blood clotting assays. J Thromb Haemost (2006) 0.94
A single genetic origin for a common Caucasian risk factor for venous thrombosis. Blood (1997) 0.93
A comparison of the abilities of plasma kallikrein, beta-Factor XIIa, Factor XIa and urokinase to activate plasminogen. Thromb Res (1983) 0.92
Binding sites for blood coagulation factor Xa and protein S involving residues 493-506 in factor Va. Protein Sci (1996) 0.92
Recent advances in the understanding of contact activation reactions. Semin Thromb Hemost (1979) 0.92
Protein S is inducible by interleukin 4 in T cells and inhibits lymphoid cell procoagulant activity. Proc Natl Acad Sci U S A (1997) 0.92
Conformational changes in aspartate transcarbamylase. II. Circular dichroism evidence for the involvement of metal ions in allosteric interactions. J Biol Chem (1973) 0.91
Molecular assembly in the contact phase of the Hageman factor system. Am J Med (1979) 0.91
Identification of divalent metal ion-dependent inhibition of activated protein C by alpha 2-macroglobulin and alpha 2-antiplasmin in blood and comparisons to inhibition of factor Xa, thrombin, and plasmin. J Biol Chem (1991) 0.91