Activated protein C inhibitor for correction of thrombin generation in hemophilia A blood and plasma

Journal of Thrombosis and Haemostasis

Volumn 9, Issue 11, 2011, Pages 2262-2267

  Brummel Ziedins, K E ^a   Whelihan, M F ^a   Rivard, G E ^b   Butenas, S ^a  

^a UNIVERSITY OF VERMONT   (United States)

^b CENTRE HOSPITALIER DE L UNIVERSITÉ DE MONTRÉAL   (Canada)

Author keywords

Activated protein C; Blood coagulation; Hemophilia A; Inhibitors; Thrombin generation; Tissue factor

Indexed keywords

[6 (LYSYLLYSYLTHREONYL[HOMO]ARGINYL)AMINO 2 [LYSYL(CARBOBENZOXY)LYSYL(CARBOBENZOXY) O BENZYL]NAPHTHALENESULFONAMIDE]; ACTIVATED PROTEIN C; BLOOD CLOTTING FACTOR 8; BLOOD CLOTTING INHIBITOR; RECOMBINANT THROMBOPLASTIN; THROMBIN; THROMBOPLASTIN; UNCLASSIFIED DRUG;

ADOLESCENT; ADULT; ARTICLE; BLOOD CLOTTING; BLOOD CLOTTING TIME; CLINICAL ARTICLE; CONTROLLED STUDY; DISEASE SEVERITY; DRUG STRUCTURE; FEMALE; HEMOPHILIA A; HUMAN; MALE; MOLECULAR WEIGHT; PARTIAL THROMBOPLASTIN TIME; PRIORITY JOURNAL;

BLOOD; BLOOD COAGULATION; BLOOD COAGULATION TESTS; CASE-CONTROL STUDIES; DRUG EVALUATION; HEMOPHILIA A; HUMANS; PARTIAL THROMBOPLASTIN TIME; PROTEIN C INHIBITOR; THROMBIN;

EID: 80055107829     PISSN: 15387933     EISSN: 15387836     Source Type: Journal    
DOI: 10.1111/j.1538-7836.2011.04504.x     Document Type: Article

References (27)

1. Gitschier J, Kogan S, Diamond C, Levinson B. Genetic basis of hemophilia A. Thromb Haemost 1991; 66: 37-9.
2. Nicholson A, Berger K, Bohn R, Carcao M, Fischer K, Gringeri A, Hoots K, Mantovani L, Schramm W, van Hout BA, Willan AR, Feldman BM. Recommendations for reporting economic evaluations of haemophilia prophylaxis: a nominal groups consensus statement on behalf of the Economics Expert Working Group of The International Prophylaxis Study Group. Haemophilia 2008; 14: 127-32.
3. Butenas S, Orfeo T, Kalafatis M, Mann KG. Peptidomimetic inhibitors for activated protein C: implications for hemophilia management. J Thromb Haemost 2006; 4: 2411-16.
4. Mann KG, Brummel-Ziedins K, Orfeo T, Butenas S. Models of blood coagulation. Blood Cells Mol Dis 2006; 36: 108-17.
5. Kalafatis M, Rand MD, Mann KG. The mechanism of inactivation of human factor V and human factor Va by activated protein C. J Biol Chem 1994; 269: 31869-80.
6. Nichols WC, Amano K, Cacheris PM, Figueiredo MS, Michaelides K, Schwaab R, Hoyer L, Kaufman RJ, Ginsburg D. Moderation of hemophilia A phenotype by the factor V R506Q mutation. Blood 1996; 88: 1183-7.
7. Lee DH, Walker IR, Teitel J, Poon MC, Ritchie B, Akabutu J, Sinclair GD, Pai M, Wu JW, Reddy S, Carter C, Growe G, Lillicrap D, Lam M, Blajchman MA. Effect of the factor V Leiden mutation on the clinical expression of severe hemophilia A. Thromb Haemost 2000; 83: 387-91.
8. Vianello F, Belvini D, Dal Bello F, Tagariello G, Zanon E, Lombardi AM, Zerbinati P, Girolami A. Mild bleeding diathesis in a boy with combined severe haemophilia B (C(10400)->T) and heterozygous factor V Leiden. Haemophilia 2001; 7: 511-14.
9. Schlachterman A, Schuettrumpf J, Liu JH, Furlan Freguia C, Toso R, Poncz M, Camire RM, Arruda VR. Factor V Leiden improves in vivo hemostasis in murine hemophilia models. J Thromb Haemost 2005; 3: 2730-7.
10. van 't Veer C, Kalafatis M, Bertina RM, Simioni P, Mann KG. Increased tissue factor-initiated prothrombin activation as a result of the Arg506->Gln mutation in factor VLEIDEN. J Biol Chem 1997; 272: 20721-9.
11. Cawthern KM, van't Veer C, Lock JB, DiLorenzo ME, Branda RF, Mann KG. Blood coagulation in hemophilia A and hemophilia C. Blood 1998; 91: 4581-92.
12. Higgins DL, Mann KG. The interaction of bovine factor V and factor V-derived peptides with phospholipid vesicles. J Biol Chem 1983; 258: 6503-8.
13. Butenas S, Orfeo T, Lawson JH, Mann KG. Aminonaphthalenesulfonamides, a new class of modifiable fluorescent detecting groups and their use in substrates for serine protease enzymes. Biochemistry 1992; 31: 5399-411.
14. Rosen S, Johansson K, Lindberg K, Dahlback B. Multicenter evaluation of a kit for activated protein C resistance on various coagulation instruments using plasmas from healthy individuals. The APC Resistance Study Group. Thromb Haemost 1994; 72: 255-60.
15. Mann KG, Whelihan MF, Butenas S, Orfeo T. Citrate anticoagulation and the dynamics of thrombin generation. J Thromb Haemost 2007; 5: 2055-61.
16. Rand MD, Lock JB, van't Veer C, Gaffney DP, Mann KG. Blood clotting in minimally altered whole blood. Blood 1996; 88: 3432-45.
17. Brummel KE, Butenas S, Mann KG. An integrated study of fibrinogen during blood coagulation. J Biol Chem 1999; 274: 22862-70.
18. Ye J, Esmon NL, Esmon CT, Johnson AE. The active site of thrombin is altered upon binding to thrombomodulin. Two distinct structural changes are detected by fluorescence, but only one correlates with protein C activation. J Biol Chem 1991; 266: 23016-21.
19. Hockin MF, Jones KC, Everse SJ, Mann KG. A model for the stoichiometric regulation of blood coagulation. J Biol Chem 2002; 277: 18322-33.
20. van 't Veer C, Golden NJ, Kalafatis M, Mann KG. Inhibitory mechanism of the protein C pathway on tissue factor-induced thrombin generation. Synergistic effect in combination with tissue factor pathway inhibitor. J Biol Chem 1997; 272: 7983-94.
21. van 'tVeer C, Golden NJ, Kalafatis M, Simioni P, Bertina RM, Mann KG. An in vitro analysis of the combination of hemophilia A and factor V(LEIDEN). Blood 1997; 90: 3067-72.
22. Campbell JE, Brummel-Ziedins KE, Butenas S, Mann KG. Cellular regulation of blood coagulation: a model for venous stasis. Blood 2010; 116: 6082-91.
23. Brummel-Ziedins KE, Whelihan MF, Gissel M, Mann KG, Rivard GE. Thrombin generation and bleeding in haemophilia A. Haemophilia 2009; 15: 1118-25.
24. De Nanteuil G, Gloanec P, Beguin S, Giesen PL, Hemker HC, Mennecier P, Rupin A, Verbeuren TJ. Low molecular weight activated protein C inhibitors as a potential treatment for hemophilic disorders. J Med Chem 2006; 49: 5047-50.
25. Prasad S, Lillicrap D, Labelle A, Knappe S, Keller T, Burnett E, Powell S, Johnson KW. Efficacy and safety of a new-class hemostatic drug candidate, AV513, in dogs with hemophilia A. Blood 2008; 111: 672-9.
26. Waters EK, Genga RM, Schwartz MC, Nelson JA, Schaub RG, Olson KA, Kurz JC, McGinness KE. Aptamer ARC19499 mediates a procoagulant hemostatic effect by inhibiting tissue factor pathway inhibitor. Blood 2011; 117: 5514-22.
27. Gringeri A, Mantovani LG, Scalone L, Mannucci PM. Cost of care and quality of life for patients with hemophilia complicated by inhibitors: the COCIS Study Group. Blood 2003; 102: 2358-63.