Factor V and thrombotic disease description of a Janus-faced protein

Arteriosclerosis, Thrombosis, and Vascular Biology

Volumn 22, Issue 4, 2002, Pages 530-538

  Nicolaes, Gerry A F ^a   Dahlbäck, Björn ^a  

^a LUND UNIVERSITY   (Sweden)

Author keywords

Activated protein C resistance; Factor V; Factor VLidien; Protein C; Thrombosis

Indexed keywords

ACTIVATED PROTEIN C; BLOOD CLOTTING FACTOR 10; BLOOD CLOTTING FACTOR 10A; BLOOD CLOTTING FACTOR 5; BLOOD CLOTTING FACTOR 5 LEIDEN; BLOOD CLOTTING FACTOR 5A; PROTEIN C;

ACTIVATED PROTEIN C RESISTANCE; ENVIRONMENTAL FACTOR; ENZYME ACTIVATION; ENZYME INACTIVATION; HEMOSTASIS; HUMAN; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; SHORT SURVEY; SIGNAL TRANSDUCTION; THROMBOEMBOLISM; THROMBOGENESIS; THROMBOSIS;

ACTIVATED PROTEIN C RESISTANCE; BLOOD COAGULATION; EUROPEAN CONTINENTAL ANCESTRY GROUP; FACTOR V; FACTOR VA; FACTOR VIII; FACTOR XA; HUMANS; PHENOTYPE; POINT MUTATION; PROTEIN C; PROTEIN S; THROMBIN; VENOUS THROMBOSIS;

EID: 0036124011     PISSN: 10795642     EISSN: None     Source Type: Journal    
DOI: 10.1161/01.ATV.0000012665.51263.B7     Document Type: Short Survey

References (107)

1. Parahaemophilia: Haemorrhagic diathesis due to absence of a previously unknown clotting factor
2. Five novel mutations in the gene for human blood coagulation factor V associated with type I factor V deficiency
3. Factor V
4. Rare coagulation disorders
5. Familial thrombophilia due to a previously unrecognized mechanism characterized by poor anticoagulant response to activated protein C: Prediction of a cofactor to activated protein C
6. Resistance to activated protein C caused by the R(506)Q mutation in the gene for factor V is a common risk factor for venous thrombosis
7. Regulation of thrombin formation by activated protein C: Effect of the factor V Leiden mutation
8. Activated protein C resistance and thrombosis: Molecular mechanisms of hypercoagulable state due to FVR506Q mutation
9. Blood coagulation
10. Risk factors in venous thromboembolism
11. Radioimmunoassay of factor V in human plasma and platelets
12. Subcellular localization and secretion of factor V from human platelets
13. Generation of coagulation factors V, XI, and XII by the isolated rat liver
14. Biosynthesis of coagulation factor V by a human hepatocellular carcinoma cell line
15. Biosynthesis of factor V in isolated guinea pig megakaryocytes
16. Biology of human megakaryocyte factor V
17. Secretable human platelet-derived factor V originates from the plasma pool
18. Cloning of cDNAs coding for the heavy chain region and connecting region of human factor V, a blood coagulation factor with four types of internal repeats
19. Complete cDNA and derived amino acid sequence of human factor V
20. Characterization of the human factor VIII gene
21. Blood coagulation factors V and VIII: Structural and functional similarities and their relationship to hemorrhagic and thrombotic disorders
22. Complete cDNA sequence of human preceruloplasmin
23. Coagulation factors V and VIII and ceruloplasmin constitute a family of structural related proteins
24. Three-dimensional model of coagulation factor Va bound to activated protein C
25. Structural investigation of the A domains of human blood coagulation factor V by molecular modeling
26. Molecular models for the two discoidin domains of human blood coagulation factor V
27. Crystal structures of the membrane-binding C2 domain of human coagulation factor V
28. Mutations in a potential phospholipid binding loop in the C2 domain of factor V affecting the assembly of the prothrombinase complex
29. The contribution of bovine factor V and factor Va to the activity of the prothrombinase
30. Activation of human factor V by factor Xa and thrombin
31. Thrombin-catalyzed activation of human coagulation factor V
32. Activation of human factor V by meizothrombin
33. The role of phospholipids and factor Va in the prothrombinase complex
34. Fatal haemorrhage and incomplete block to embryogenesis in mice lacking coagulation factor V
35. The mechanism of inactivation of human factor V and human factor Va by activated protein C
36. Peptide bond cleavages and loss of functional activity during inactivation of factor Va and factor VaR506Q by activated protein
37. R506Q by activated protein C
38. Protein S as an in vivo cofactor to activated protein C in prevention of microarterial thrombosis in rabbits
39. Antithrombotic effects of activated protein C and protein S in a rabbit model of microarterial thrombosis
40. Inherited thrombophilia
41. The role of the membrane in the inactivation of factor Va by plasmin: Amino acid region 307-348 of factor V plays a critical role in factor Va cofactor function
42. Thrombophilia: The discovery of activated protein C resistance
43. Inherited resistance to activated protein C is corrected by anticoagulant cofactor activity found to be a property of factor V
44. Factor V and protein S as synergistic cofactors to activated protein C in degradation of factor VIIIa
45. Mutation in blood coagulation factor V associated with resistance to activated protein C
46. Activated protein C resistance caused by Arg506Gln mutation in factor Va
47. 506 of factor V
48. Linkage between inherited resistance to activated protein C and factor V gene mutation in venous thrombosis
49. World distribution of factor V Leiden
50. A single genetic origin for a common Caucasian risk factor for venous thrombosis
51. Influence of factor V and factor Va on APC-induced cleavage of human factor VIII
52. Factor V enhances the cofactor function of protein S in the APC-mediated inactivation of factor VIII: Influence of the factor VR506Q mutation
53. Comparison of activated protein C/protein S-mediated inactivation of human factor VIII and factor V
54. Cleavage of factor V at Arg 506 by activated protein C and the expression of anticoagulant activity of factor V
55. Effect of activated protein C on thrombin generation and on the thrombin potential in plasma of normal and APC resistant individuals
56. The C-terminal region of the factor V B-domain is crucial for the anticoagulant activity of factor V
57. Characterization of two forms of human factor Va with different cofactor activities
58. Human factor Va1 and factor Va2: Properties in the procoagulant and anticoagulant pathways
59. Partial glycosylation at asparagine-2181 of the second C-type domain of human factor V modulates assembly of the prothrombinase complex
60. Partial glycosylation of Asn218I in human factor V as a cause of molecular and functional heterogeneity: Modulation of glycosylation efficiency by mutagenesis of the consensus sequence for N-linked glycosylation
61. Pathogenesis of venous thrombosis
62. Venous thrombosis: A multicausal disease
63. The epidemiology, pathogenesis and natural history of venous thrombosis
64. A population-based perspective of the hospital incidence and case-fatality rates of deep vein thrombosis and pulmonary embolism: The Worcester DVT Study
65. A prospective study of the incidence of deep-vein thrombosis within a defined urban population
66. Thrombophilia as a multigenic disorder
67. The protein C anticoagulant system: Inherited defects as basis for venous thrombosis
68. Genetic aspects of venous thrombosis
69. Thrombophilia as a multigenic disease
70. High risk of thrombosis in patients homozygous for factor V Leiden (activated protein C resistance)
71. Venous thrombosis due to a poor anticoagulant response to activated protein C: Leiden Thrombophilia Study
72. A study of protein S antigen levels in 3788 healthy volunteers: Influence of age, sex and hormone use, and estimate for prevalence of deficiency status
73. Low prevalence of factor V:Q506 in 41 patients with isolated pulmonary embolism
74. Activated protein C resistance, factor V Leiden, and retinal vessel occlusion
75. The risk of recurrent venous thromboembolism in carriers and non-carriers of the G1691A allele in the coagulation factor V gene and the G20210A allele in the prothrombin gene: DURAC Trial Study Group Duration of Anticoagulation
76. Risk of recurrent venous thromboembolism in patients with the factor V Leiden (FVR506Q) mutation: Effect of warfarin and prediction by precipitating factors: East Anglian Thrombophilia Study Group
77. The risk of recurrent venous thromboembolism in patients with and without factor V Leiden
78. The risk of recurrent venous thromboembolism in patients with an Arg506→Gln mutation in the gene for factor V (factor V Leiden)
79. Geographic distribution of the 20210 G to A prothrombin variant
80. Activated protein C resistance as an additional risk factor for thrombosis in protein C-deficient families
81. Resistance to activated protein C as an additional genetic risk factor in hereditary deficiency of protein S
82. Factor V Leiden: An additional risk factor for thrombosis in protein S deficient families
83. Factor V Leiden (FV R506Q) in families with inherited antithrombin deficiency
84. Study of the prothrombin gene 20201 GA variant in FV:Q506 carriers in relationship to the presence or absence of juvenile venous thromboembolism
85. Increased risk of venous thrombosis in oral-contraceptive users who are carriers of factor V Leiden mutation
86. Oral contraceptives and venous thromboembolism: Acquired APC resistance
87. Low-dose oral contraceptives and acquired resistance to activated protein C: A randomised cross-over study
88. Hormone replacement therapy and acquired resistance to activated protein C: Results of a randomized, double-blind, placebo-controlled trial
89. Factor V Cambridge: A new mutation (Arg306→Thr) associated with resistance to activated protein C
90. A novel mutation of Arg306 of factor V gene in Hong Kong Chinese
91. The APC-resistance pattern of recombinant factor V Cambridge and Factor V Hong Kong
92. A factor V genetic component differing from factor V R506Q contributes to the activated protein C resistance phenotype
93. Detection of new polymorphic markers in the factor V gene: Association with factor V levels in plasma
94. The factor V gene A4070G mutation and the risk of venous thrombosis
95. Coinheritance of the HR2 haplotype in the factor V gene confers an increased risk of venous thromboembolism to carriers of factor V R506Q (factor V Leiden)
96. The factor V R2 allele: Risk of venous thromboembolism, factor V levels and resistance to activated protein C
97. Functional properties of factor V and factor Va encoded by the R2-gene
98. Mutations in the R2 FV gene affect the ratio between the two FV isoforms in plasma
99. Recurrent thrombosis due to compound heterozygosity for factor V Leiden and factor V deficiency
100. "Pseudo homozygous" activated protein C resistance due to double heterozygous factor V defects (factor V Leiden mutation and type I quantitative factor V defect) associated with thrombosis: Report of two cases belonging to two unrelated kindreds
101. Molecular characterization of a type I quantitative factor V deficiency in a thrombosis patient that is "pseudo homozygous" for activated protein C resistance
102. Phenotypic homozygous activated protein C resistance associated with compound heterozygosity for Arg506Gln (factor V Leiden) and His1299Arg substitutions in factor V
103. Pseudo-homozygous activated protein C resistance due to coinheritance of heterozygous factor V Leiden mutation and type I factor V deficiency: Variable expression when analyzed by different activated protein C resistance functional assays
104. A novel factor V null mutation detected in a thrombophilic patient with pseudohomozygous APC resistance and in an asymptomatic unrelated subject
105. A reduced sensitivity for activated protein C in the absence of factor V Leiden increases the risk of venous thrombosis
106. Factor V antigen levels and venous thrombosis: Risk profile, interaction with factor V Leiden, and relation with factor VIII antigen levels
107. Clinical and laboratory manifestations of anti-factor V antibodies