Deficient APC-cofactor activity of protein S Heerlen in degradation of factor Va Leiden: a possible mechanism of synergism between thrombophilic risk factors

Venous thrombosis: a multicausal disease

1999 • 1,570 citations

Activated protein C resistance as an additional risk factor for thrombosis in protein C-deficient families

1994 • 446 citations

Different Risks of Thrombosis in Four Coagulation Defects Associated With Inherited Thrombophilia: A Study of 150 Families

1998 • 437 citations

Resistance to activated protein C as an additional genetic risk factor in hereditary deficiency of protein S

1995 • 314 citations

Factor V and protein S as synergistic cofactors to activated protein C in degradation of factor VIIIa.

1994 • 307 citations

Inhibition of protein Ca cofactor function of human and bovine protein S by C4b-binding protein.

1986 • 296 citations

The protein C anticoagulant system: Inherited defects as basis for venous thrombosis

1995 • 286 citations

Glycoproteins: glycan presentation and protein-fold stability

1999 • 285 citations

Changes in the plasma levels of vitamin K-dependent proteins C and S and of C4b-binding protein during pregnancy and oral contraception

1988 • 259 citations

Single and Combined Prothrombotic Factors in Patients With Idiopathic Venous Thromboembolism

1999 • 258 citations

Effects of Protein S and Factor Xa on Peptide Bond Cleavages during Inactivation of Factor Va and Factor VaR506Q by Activated Protein C

1995 • 233 citations

Risk factors for venous thrombosis: prevalence, risk, and interaction.

1997 • 221 citations

Binding of protein S to factor Va associated with inhibition of prothrombinase that is independent of activated protein C.

1993 • 220 citations

Isolation and sequence of the cDNA for human protein S, a regulator of blood coagulation.

1986 • 204 citations

Evaluation of the relationship between protein S and C4b-binding protein isoforms in hereditary protein S deficiency demonstrating type I and type III deficiencies to be phenotypic variants of the same genetic disease

1995 • 168 citations

Inherited predisposition to thrombosis

1993 • 166 citations

Human protein S inhibits prothrombinase complex activity on endothelial cells and platelets via direct interactions with factors Va and Xa.

1994 • 157 citations

Risk of Venous Thromboembolism and Clinical Manifestations in Carriers of Antithrombin, Protein C, Protein S Deficiency, or Activated Protein C Resistance

1999 • 156 citations

Factor V Leiden: An Additional Risk Factor for Thrombosis in Protein S Deficient Families?

1995 • 152 citations

Gene-Gene and Gene-Environment Interactions Determine Risk of Thrombosis in Families With Inherited Antithrombin Deficiency

1999 • 134 citations

Reevaluation of total, free, and bound protein S and C4b-binding protein levels in plasma anticoagulated with citrate or hirudin

1992 • 126 citations

Characterization of functionally important domains in human vitamin K-dependent protein S using monoclonal antibodies.

1990 • 112 citations

Binding of bovine coagulation factor X_a to platelets

1978 • 106 citations

Comparison of activated protein C/protein S-mediated inactivation of human factor VIII and factor V

1996 • 105 citations

Clarification of the Risk for Venous Thrombosis Associated with Hereditary Protein S Deficiency by Investigation of a Large Kindred with a Characterized Gene Defect

1998 • 103 citations

Genetic and Phenotypic Analysis of a Large (122-Member) Protein S–Deficient Kindred Provides an Explanation for the Familial Coexistence of Type I and Type III Plasma Phenotypes

1997 • 103 citations

Heerlen polymorphism of protein S, an immunologic polymorphism due to dimorphism of residue 460

1990 • 101 citations

New Insights into How Blood Clots: Implications for the Use of APTT and PT as Coagulation Screening Tests and in Monitoring of Anticoagulant Therapy

1999 • 96 citations

Protein C and protein S deficiencies.

1997 • 87 citations

Resistance to activated protein C as risk factor for thrombosis: molecular mechanisms, laboratory investigation, and clinical management.

1997 • 86 citations

Inhibition of the intrinsic factor X activating complex by protein S: evidence for a specific binding of protein S to factor VIII

1995 • 85 citations

A Common 4G Allele in the Promoter of the Plasminogen Activator Inhibitor-1 (PAI-1) Gene as a Risk Factor for Pulmonary Embolism and Arterial Thrombosis in Hereditary Protein S Deficiency

1998 • 76 citations

Three novel mutations in five unrelated subjects with hereditary protein S deficiency type I.

1994 • 72 citations

Cleavage Requirements for Activation of Factor V by Factor Xa

1997 • 71 citations

The Ser 460 to Pro substitution of the protein S alpha (PROS1) gene is a frequent mutation associated with free protein S (type IIa) deficiency

1995 • 69 citations

Molecular basis for protein S hereditary deficiency: Genetic defects observed in 118 patients with type I and type IIa deficiencies1

1996 • 62 citations

Protein C Nagoya, an elongated mutant of protein C, is retained within the endoplasmic reticulum and is associated with GRP78 and GRP94

1996 • 62 citations

Protein S deficiency.

1997 • 58 citations

Protein S Deficiency

1997 • 51 citations

Characterization of mini-protein S, a recombinant variant of protein S that lacks the sex hormone binding globulin-like domain

1998 • 49 citations

The SHBG‐like region of protein S is crucial for factor V‐dependent APC‐cofactor function

1998 • 48 citations

Procoagulant and anticoagulant properties of coagulation factor V: Factor V Leiden (APC resistance) causes hypercoagulability by dual mechanisms

1999 • 47 citations

Binding Site for C4b-Binding Protein in Vitamin K-Dependent Protein S Fully Contained in Carboxy-Terminal Laminin-G-type Repeats. A Study Using Recombinant Factor IX-Protein S Chimeras and Surface Plasmon Resonance

1997 • 42 citations

Amino Acid Residues in Thrombin-sensitive Region and First Epidermal Growth Factor Domain of Vitamin K-dependent Protein S Determining Specificity of the Activated Protein C Cofactor Function

1998 • 40 citations

A New Direct, Fast and Quantitative Enzyme-linked Ligandsorbent Assay for Measurement of Free Protein S Antigen

1998 • 40 citations

C-terminal Residues 621–635 of Protein S Are Essential for Binding to Factor Va

1999 • 37 citations

Both G‐type domains of protein S are required for the high‐affinity interaction with C4b‐binding protein

1999 • 36 citations

Protein S gene analysis reveals the presence of a cosegregating mutation in most pedigrees with type I but not type III PS deficiency

1999 • 35 citations

Expression and Functional Characterization of Chimeras Between Human and Bovine Vitamin‐K‐dependent Protein‐S‐defining Modules Important for the Species Specificity of the Activated Protein C Cofactor Activity

1995 • 35 citations

Molecular basis of a hereditary type I protein S deficiency caused by a substitution of Cys for Arg474

1996 • 35 citations

Clinical features of thrombophilia in families with gene defects in protein C or protein S combined with factor V Leiden

1998 • 32 citations

Familial protein S deficiency with a variant protein S molecule in plasma and platelets

1989 • 32 citations

Protein S secretion differences of missense mutants account for phenotypic heterogeneity

2000 • 31 citations

Mutations causing coagulation factor XIII subunit A deficiency: characterization of the mutant proteins after expression in yeast

1995 • 30 citations

A Plasma Coagulation Assay for an Activated Protein C-independent Anticoagulant Activity of Protein S

1998 • 29 citations

The Effect of N-linked Glycosylation on Molecular Weight, Thrombin Cleavage, and Functional Activity of Human Protein S

1997 • 28 citations

Absence of Linkage Between Type III Protein S Deficiency and the PROS1 and C4BP Genes in Families Carrying the Protein S Heerlen Allele

1997 • 27 citations

Homozygosity for the Protein S Heerlen Allele Is Associated with Type I PS Deficiency in a Thrombophilic Pedigree with Multiple Risk Factors

2000 • 26 citations

New Direct Assay of Free Protein S Antigen Applied to Diagnosis of Protein S Deficiency

1996 • 24 citations

A Mutation of the Active Protein S Gene Leading to an EGF1-Lacking Protein in a Family With Qualitative (Type II) Deficiency

1998 • 23 citations

Poor Relationship between Phenotypes of Protein S Deficiency and Mutations in the Protein S Alpha Gene

1999 • 22 citations

In Vitro Characterisation of Two Naturally Occurring Mutations in the Thrombin-sensitive Region of Anticoagulant Protein S

1999 • 13 citations