Factor V enhances the cofactor function of protein S in the APC-mediated inactivation of factor VIII: Influence of the factor V(R506Q) mutation

Thrombosis and Haemostasis

Volumn 76, Issue 2, 1996, Pages 208-214

  Varodi K ^a   Rosing, J ^b   Tans, G ^b   Pabinger, I ^c   Keil, B ^a   Schwarz, H P ^a,d  

^a Research Laboratories of Immuno AG   (Austria)

^b MAASTRICHT UNIVERSITY   (Netherlands)

^c UNIVERSITY HOSPITAL INNSBRUCK   (Austria)

^d BAXTER HEALTHCARE CORPORATION   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

ACTIVATED PROTEIN C; BINDING PROTEIN; BLOOD CLOTTING FACTOR 10A; BLOOD CLOTTING FACTOR 5; BLOOD CLOTTING FACTOR 8; CHROMOGENIC SUBSTRATE; COMPLEMENT COMPONENT C4B; MUTANT PROTEIN; PROTEIN S;

ARTICLE; BLOOD CLOTTING FACTOR 5 DEFICIENCY; COMPLEX FORMATION; CONTROLLED STUDY; GENE MUTATION; HETEROZYGOTE; HOMOZYGOSITY; HUMAN; MAJOR CLINICAL STUDY; PLASMA FACTOR; PRIORITY JOURNAL; PROTEIN PURIFICATION; PROTEIN S DEFICIENCY;

EID: 0029792536     PISSN: 03406245     EISSN: None     Source Type: Journal    
DOI: 10.1055/s-0038-1650556     Document Type: Article

References (30)

1. Esmon CT, Schwarz HP. An update on clinical and basic aspects of the protein C anticoagulant pathway. Trends in Cardiovasc Med 1995; 5: 141-8.
2. Dreyfus M, Magny JF, Bridey F, Schwarz HP, Planche C, Dehan M, Tchernia G. Treatment of Homozygous Protein C Deficiency and Neonatal Purpura Fulminans with a Purified Protein C Concentrate. N Engl J Med 1991; 325: 1565-8.
3. Griffin JH, Evatt BL, Zimmerman TS, Kleiss AJ, Wideman C. Deficiency of protein C in congenital thrombotic disease. J Clin Invest 1981; 68: 1370-3.
4. Walker FJ. Regulation of activated protein C by protein S. J Biol Chem 1981; 256: 11128-31.
5. Amer L, Kisiel W, Searles RP, Williams RC. Impairment of the protein C anticoagulant pathway in a patient with systemic lupus erythematosus, anticardiolipin antibodies and thrombosis. Thromb Res 1990; 57: 247-58.
6. Marciniak E, Romond EH. Impaired catalytic function of activated protein C: A new in vitro manifestation of lupus anticoagulant. Blood 1989; 74: 2426-32.
7. Dahlbäck B, Carlsson M. Factor VIII defect associated with familial thrombophilia. Thromb Haemost 1991; 65: 658.
8. Dahlbäck B, Hildebrand B. Inherited resistance to activated protein C is corrected by anticoagulant cofactor activity found to be a property of factor V. Proc Natl Acad Sci USA 1994; 91: 1396-400.
9. Dahlbäck B, Carlsson M, Svensson PJ. 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. Proc Natl Acad Sci USA 1993; 90: 1004-8.
10. Koster T, Rosendaal FR, de Ronde H, Briët E, Vandenbroucke JP, Bertina RM. Venous thrombosis due to poor anticoagulant response to activated protein C: Leiden thrombophilia study. Lancet 1993; 342: 1503-6.
11. Svensson PJ, Dahlbäck B. Resistance to activated protein C as a basis for venous thrombosis. N Engl J Med 1994; 330: 517-22.
12. Griffin JH, Evatt BL, Wideman C, Fernandez JA. Anticoagulant protein C pathway defective in majority of thrombophilic patients. Blood 1993; 82: 1989-93.
13. Dahlbäck B. Physiological anticoagulation. Resistance to activated protein C and venous thromboembolism. J Clin Invest 1994; 94: 923-7.
14. Bertina RM, Koeleman BP, Koster T, Rosendaal FR, Dirven RJ, de Ronde H, van der Velden PA, Reitsma PH. Mutation in blood coagulation factor V associated with resistance to activated protein C. Nature 1994; 369: 64-7.
15. Greengard JS, Sun X, Xu X, Fernandez JA, Griffin JH, Evatt B. Activated protein C resistance caused by Arg506Gln mutation in factor Va. The Lancet 1994; 343: 1361-2.
16. 506 of factor V. The Lancet 1994; 343: 1535-6.
17. Zöller B, Svensson PJ, He X, Dahlbäck B. Identification of the same factor V gene mutation in 47 out of 50 thrombosis prone families with inherited resistance to activated protein C. J Clin Invest 1994; 94: 2521-4.
18. Shen L, Dahlbäck B. Factor V and protein S as synergistic cofactors to activated protein C in degradation of factor VIIIa. J Biol Chem 1994, 269: 18735-8.
19. Váradi K, Rosing J, Tans G, Schwarz HP. Influence of factor V and factor Va on APC-induced cleavage of human factor VIII. Thromb Haemost 1995; 73: 730.
20. Beauchamp, NJ, Daly ME, Hampton KK, Cooper PC, Preston FE, Peake IR. High prevalence of a mutation in the factor V gene within the U.K. population: Relationship to activated protein C resistance and familial thrombosis. Br J Haematol 1994; 88: 219-22.
21. Tans G, Rosing J, Thomassen MCLGD, Heeb MJ, Zwaal RFA, Griffin JH. Comparison of anticoagulant and procoagulant activities of stimulated platelets and platelet-derived microparticles. Blood 1991; 77: 2641-8.
22. Rosing J, Bakker HM, Thomassen MCLGD, Hemker HC, Tans G. Characterization of two forms of human factor Va with different cofactor activities. J Biol Chem 1993; 268: 21130-6.
23. Lang H, Oberreither M, Moritz B. A new chromogenic factor VIII:C - Assay based on a system of human proteins. (Abstract) Thromb Haemost 1991; 65: 943.
24. Váradi K, Moritz B, Lang H, Bauer K, Preston E, Peake I, Rivard GE, Keil B, Schwarz HP. A chromogenic assay for activated protein C resistance. Br J Haematol 1995; 90: 884-91.
25. Fay PJ, Smudzin TM, Walker FJ. Activated protein C-catalyzed inactivation of human factor VIII and factor VIIIa. J Biol Chem 1991; 266: 20139-45.
26. Dahlbäck B. Inhibition of protein Ca cofactor function of human and bovine protein S by C4b-binding protein. J Biol Chem 1986; 261: 12022-7.
27. Miller SP. Coagulation dynamics in factor V deficiency: A family study, with a note on the occurrence of thrombophlebitis. Thromb Diath Haemorrh 1965; 13: 500-15.
28. Fowler WE, Fay PJ, Arvan DS, Marder VJ. Electron microscopy of human Factor V and Factor VIII: Correlation of morphology with domain structure and localization of Factor V activation fragments. Proc Natl Acad Sci USA 1990; 87: 7648.
29. Dahlbäck B. A new model for coagulation Factor V suggesting a unique mechanism of activation. Scand J Clin Lab Invest 1988; 191: 47.
30. R506Q by activated protein C. J Biol Chem 1995; 270: 27852-8.