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12. Saenko, E.L., Shima, M., and Sarafanov, A. Role of activation of the coagulation factor VIII in interactions with von Willebrand factor, phospholipid, and functioning within the factor Xase complex. Invited Review, Trends Cardiovasc. Med., 9:185-192 (1999).

13. Nogami, K., Shima, M., Nakai, H., Tanaka, I., Suzuki, H., Morichika, S., Shibata, M., Saenko, E.L., Scandella, D., Giddings, J.C., and Yoshioka, A. Identification of a factor VIII peptide, residues 2315-2330, which neutralizes human factor VIII C inhibitor alloantibodies: requirement of Cys2326 and Glu2327 for maximum effect. Br. J.

Haematol., 107: 196-203 (1999).

14. Nogami, K., Shima, M., Hosokawa, K., Suzuki, T., Koide, T., Saenko, E.L., Scandella, D., Shibata, M., Kamisue, S., Tanaka, I., and A. Yoshioka. Role of factor VIII C2 domain in factor VIII binding to factor Xa. J. Biol. Chem., 274: 31000-31007 (1999).

15. Saenko, E.L., Loster, K., Josic, D., and Sarafanov, A. Effect of von Willebrand factor and its proteolytic fragments on kinetics of interaction between the light chain and heavy chains of human factor VIII. Thromb. Res., 96: 355-363 (1999).

16. Saenko E.L., Yakhyaev, A.V., Mikhailenko I., Strickland D., and Sarafanov, A. Role of the low density lipoprotein-related protein receptor in mediation of factor VIII catabolism. J. Biol. Chem., 274: 37685-37692 (1999).

17. Moreau, A., Lacroix-Desmazes, S., Stieltjes, N., Saenko, E.L., Kaveri, S.V., DOrion, R., Sultan, Y., Scandella, D., Kazatchkine, D. Antibodies to the FVIII light chain that neutralize FVIII procoagulant activity are present in plasma of non-responder patients with severe hemophilia A and in normal polyclonal human IgG. Blood, 95:

3435-3441 (2000).

18. Nogami, K., Shima, M., Hosokawa, K., Nagata, M., Koide, T., Saenko, E.L., Tanaka, I., Shibata, M., and Yoshioka, A. Factor VIII C2 domain contains the thrombin binding site responsible for thrombin-catalyzed cleavage at Arg1689. J.Biol.Chem., 275:

25774-25780 (2000).

19. Mondorf, W., Klinge, J., Luban, N., Bray, G., Saenko, E.L., Scandella, D. Low factor VIII recovery in Hemophilia A patients without inhibitor titer is not due to the presence of anti factor VIII antibodies undetectable by the Bethesda assay. Haemophilia, 7: 13-19 (2001).

20. Pipe, S.W., Saenko, E.L., Eickhorst, A.N., Kemball-Cook, G., and Kaufman, R.J. Hemophilia A mutations associated with one-stage/two-stage activity discrepancy disrupt protein-protein interactions within the triplicated A domains of thrombin activated factor VIIIa. Blood, 97: 685-691 (2001).

21. Nogami, K., Shima, M., Giddings, J.C., Hosokawa, K., Kamisue, S., Morichika, S., Suzuki, H., Shibata, M., Saenko, E.L., Tanaka, I., Yoshioka, A.

Circulating factor VIII immune complexes in the patients with type 2 acquired hemophilia A and protection from activated protein C-mediated proteolysis. Blood, 97:

669-677 (2001).

22. Ananyeva, N., Kouiavskaia, D., Shima M., Saenko, E.L. Catabolism of the coagulation factor VIII. Can we prolong lifetime of fVIII in circulation? Trends Cardiovasc. Med., 11:252-257 (2001).

23. Scandella, D., Nakai, H. Felch, M., Mondorf, W., Scharrer, I., Hoyer, L.W., and Saenko, E.L. In hemophilia A and autoantibody inhibitor patients the factor VIII A domain and light chain are most immunogenic. Thromb. Res., 101: 377-385 (2001).

24. Saenko, E.L., Josic, Dj., Stadler, M., Sarafanov, A., Yow-Pin Lim, Ananyeva, N., Schwinn, H. Molecular modifications in factor VIII concentrates produced from different plasma pools. Thromb. Res., 101: 501-511 (2001).

25. Sarafanov, A., Ananyeva, N., Shima, M., and Saenko, E.L. Cell surface heparan sulfate proteoglycans participate in factor VIII catabolism mediated by low density lipoprotein receptor-related protein (LRP). J.Biol.Chem., 276: 11970- (2001).

26. Nogami K., Shima, M., Nishiya K., Sakurai, Y., Tanaka, I., Gidding J.C., Saenko, E.L., Yoshioka, A. Human factor VIII inhibitor alloantibodies with a C2 epitope inhibit factor Xa-catalyzed factor VIII activation: a new anti-factor VIII inhibitory mechanism. Thromb. Haemost., 87: 459-465 (2002).

27. Nogami K., Shima, M., Nishiya K., Hosogawa, K., Nagata, M., Saenko, E.L., Tanaka, I., Yoshioka, A. Anticoagulant effects of a synthetic peptide containing residues Thr2253-Gln2270 within factor VIII C2 domain that effectively inhibits factor Xa-catalyzed factor VIII activation. Br. J. Haematol., 116: 868-874 (2002).

28. Saenko, E.L., Ananyeva, N.M., Kouiavskaia, D.V., Khrenov, A.V., Anderson, J.A.M., Shima, M., Qian, J. and Scott, D. Hemophilia A: effects of inhibitory antibodies on factor VIII functional interactions and approaches to prevent their action, Haemophilia, 8:1-11 (2002).

29. Nogami K., Shima, M., Nishiya K., Hosogawa, K., Saenko, E.L., Sakurai, Y., Shibata, M., Tanaka, I., Yoshioka, A. A novel mechanism of factor VIII protection by von Willebrand factor from activated protein C-catalyzed inactivation. Blood, 99: 3393 3398 (2002).

30. Hakeos, W.H., Miao, H., Sirachainan, N., Kemball-Cook, G., Saenko, E.L., Kaufman, R., and Pipe, S.W., and. Hemophilia A mutations within the factor VIII A2-A subunit interface destabilize VIIIa and cause one-stage/two-stage activity discrepancy, Thromb. Hemost., 88: 781-787 (2002).

31. Saenko, E.L., Ananyeva, N.M., Tuddenham, E.G. D., and Kemball-Cook, G.

Factor VIII novel insights into form and function. Br. J. Haematol., 119:323- (2002).

32. Saenko, E.L., Ananyeva, N.M., Kouiavskaia, D., Schwinn, H., Josic, D., Shima, M., Hauser, C., and Pipe, S.W. Molecular defects in coagulation factor VIII and their impact on factor VIII function. Vox Sanguinis, 83:89-96 (2002).

33. Klinge, J., Ananyeva, N.M., Hauser, C., and Saenko, E.L. Hemophilia from basic science to clinical practice, Seminars Thromb. Haemost.., 28: 309-321 (2002).

34. Saenko, E.L., Ananyeva, N.M., Moayeri, M., Ramezani, A., Hawley, R.G.

Development of improved factor VIII molecules and new gene transfer approaches for hemophilia A. Current Gene Therapy, 3:27-41 (2003).

35. Mhle, C., Schulz-Drost, S., Khrenov, A., Saenko, E.L., Klinge, J., Schneider, H.: Epitope mapping of polyclonal clotting factor VIII-inhibitory antibodies using phage display. Thromb. Haemost., 91:619-625 (2004).

36. Saenko, E.L., Ananyeva, N.M., Shima, M., Hauser, C. and Pipe, S.W. The future of recombinant coagulation factors. J. Thromb. Haemost., 1:922-930 (2003).

37. Sarafanov A. and Saenko E.L. High-throughput optimization of protein expression in the baculovirus system based on determination of relative expression efficiency of viral stocks. Anal. Biochem., 328:98-100 (2004).

38. Ananyeva N., Lacroix-Desmazes S., Hauser, C., Ovanesov, M.V., Khrenov, A.V., and Saenko E.L. Inhibitors in hemophilia A: mechanisms of inhibition, management and perspectives. Blood Coagul. Fibrinol., 15:109-124 (2004).

39. Ananyeva, N., Khrenov, A., Darr, F., Summers, R., Sarafanov, A., and Saenko E. Treating haemophilia A with recombinant blood factors: a comparison. Expert Opin.

Pharmacother., 5:1061-1070 (2004).

40. Oldenburg, J., Ananyeva N., and Saenko E. Molecular basis of haemophilia A.

Haemophilia, 10 (Suppl. 4): 133-139 (2004).

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VIII. . ., . 50, 5, . 29 34 (2005).

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50, 6, . 21-29 (2005).

43. Sarafanov, A., Makogonenko, E., Pechik, I., Radtke, K.-P., Khrenov, A., Ananyeva, N., Strickland, D. and E. Saenko Identification of coagulation factor VIII A domain residues forming the binding epitope for low-density lipoprotein receptor-related protein, Biochemistry, 45:1829-1840 (2006).

44. Saenko, E.L. and Pipe, S.W. Strategies toward a longer-acting factor VIII.

Haemophilia, 12 (Suppl. 30):42-51 (2006).

45. Suzuki H., Shima M., Nogami K., Sakurai Y., Saenko E.L., and A. Yoshioka.

Factor V C2 domain contains a major thrombin-binding site responsible for thrombin catalyzed factor V activation, J. Thromb. Haemost., 4:1354-1360 (2006).

46. Khrenov, A., Ananyeva, N., and Saenko, E. Role of the B-domain in proteolytic inactivation of activated coagulation factor VIII by activated protein C and activated factor X. Blood Coagul. Fibrinol., 17: 379-388 (2006).

47. Saenko, E. and Ananyeva, N. Receptor-mediated clearance of FVIII:

implications for pharmacokinetic studies in individuals with Haemophilia, Haemophilia, 12 (Suppl. 4), 15-22 (2006).

48. Sbastien Lacroix-Desmazes, Jagadeesh Bayry, Joseph Reinbolt, Valrie Roussel-Robert, Evgueni L. Saenko, Johan Hoebeke, Michel D Kazatchkine and Srini V Kaveri. Catalytic IgG from patients with Hemophilia A inactivate therapeutic factor VIII. Journal of Immunology, 177:1355-1363 (2006).

49. Muhle, C., Klinge, J., Khrenov, A. V, Saenko, E. L. and Schneider, H.

Spectrum of antigenic FVIII domains recognized by antibodies from haemophiliacs.

Haemostaseologie 28 (Suppl. 1), 1-2 (2006).

50. Panteleev, M.A., Ananyeva, N.M., Ataullakhanov, F.I., and Saenko, E.L.

Mathematical models of blood coagulation and platelet adhesion: clinical applications, Current Pharmaceutical Design, 13: 1457-1467 (2007).

51. Sarafanov, A.G., Makogonenko, E.M., Andersen, O.M., Mikhailenko, I.A., Ananyeva, N.M., Khrenov, A.V., Shima, M., Strickland, D.K., and Saenko, E.L.

Localization of the low-density lipoprotein receptor-related protein regions involved in binding to the A2 domain of coagulation factor VIII. Thromb. Haemost., 98:1170- (2007).

52. Nogami K., Saenko, E.L., Takeyama, M., Gidding, J.C., Yoshioka, A., Shima, M. Identification of a thrombin-interactive site within the FVIII A2 domain that is responsible for the cleavage at Arg372. Br. J. Haematol., 140:433-443 (2008).

53. Ananyeva, N.M., Makogonenko, Y.M., Kouiavskaia, D.V., Ruiz, J., Limburg, V., Meijer, A.B., Khrenov, A.V., Shima, M., Strickland, D.K., Saenko E.L. The binding sites for the very low density lipoprotein receptor and low-density lipoprotein receptor related protein are shared within coagulation factor VIII. Blood Coagul. Fibrinol., 19:166-177 (2008).

54. Nogami, K., Nishiya, K., Saenko, E.L., Takeyama, M., Tanaka, I., Yoshioka, A., Shima, M. Identification of a plasmin-interactive site within the A2 domain of the factor VIII heavy chain. Biochim. Biophys. Acta. 1784:753-63 (2008).

55. Ananyeva, N.M., Makogonenko, Y.M., Sarafanov, A.G., Pechik, I.V., Gorlatova, N., Radtke, K.-R., Shima, M., Saenko, E.L.. Interaction of coagulation factor VIII with members of the low-density lipoprotein receptor family follows common mechanism and involves consensus residues within the A2 binding site 484-509. Blood Coagul. Fibrinol., 19:543-55 (2008).

56. Takeyama, M., Nogami, K., Saenko, E.L., Soeda, T., Nishiya, K., Ogiwara, K., Yoshioka, A., Shima, M. Protein S down-regulates factor Xase activity independent of activated protein C: specific binding of factor VIII(a) to protein S inhibits interactions with factor IXa. Br. J. Haematol., 143:409-20 (2008).

57. Nogami K., Nishiya K., Saenko E.L., Takeyama M., Ogiwara, K., Yoshioka A., Shima M. Identification of a plasmin-interactive sites in the light chain of factor VIII responsible for proteolytic cleavage at Lys36. J. Biol. Chem., 284:6934-45 (2009).

58. Takeyama M, Nogami K, Saenko EL, Nishiya K, Ogiwara K, Shima M.

Identification of a protein S-interactive site within the A2 domain of the factor VIII heavy chain. Thromb. Haemost., 102:645-55 (2009).

59. Ananyeva NM, Lee TK, Jain N, Shima M, Saenko EL. Inhibitors in hemophilia A: advances in elucidation of inhibitory mechanisms and in inhibitor management with bypassing agents. Semin. Thromb. Haemost., 35:735-51 (2009).

60. Marlar, R.A., Saenko, E. Editorial: critical need for pediatric hematologists to specialize in hemostasis and thrombosis. Blood Coagul. Fibrinol.., 21 (Suppl 1):1- (2010).

1. Saenko, E.L., Scandella, D. The role of the acidic region of the factor VIII light chain in its binding to von Wilebrand factor, XVIth congress of the ISTH, Florence, Italy, June 6-12, 1997. Blood (1997), bstract 571.

2. Sarafanov, A.G., Ananyeva, N.M., and Saenko, E.L. Activation and Inactivation of the Intrinsic Factor Xase Complex Assembled on Plasma Lipoproteins. 41st Annual Meeting of the American Society of Hematology, New Orleans, Louisiana, December 3 7, 1999. Blood (1999), bstract 457a.

3. Saenko, E.L., Yakhyaev, A.V., Mikhailenko, I., Strickland, D., and Sarafanov, A. Role of the low density lipoprotein-related protein receptor in mediation of catabolism of coagulation factor VIII. XVIIth Congress of the International Society on Thrombosis and Haemostasis, Washington, DC, USA, September 15-21, 1999.Thromb. Haemost., Supplement, (1999), P.1.

4. Sarafanov, A., Behr, E., and Saenko, E.L. Comparison of coagulation factor VIII binding properties of the two phosphatidylserine-containing surfaces formed on HPA and L1 biosensor chips. International BIAsymposium-1999. November 7-10, 1999, San Francisco CA. Biotechniques (1999), 54: 112.

5. Saenko, E.L., Ananyeva, N.M., Josic, D., Schwinn, H., and Sarafanov, A.G.

Regulation of the Intrinsic Factor Xase Complex by Plasma Lipoproteins. 16th International Congress on Thrombosis, Porto, Portugal, May 5-8, 2000. Haemostasis, Supplement 1, P. 74 (2000).

6. Saenko, E.L., Sarafanov, A.G., Ananyeva, N.M. Role of cell surface heparan sulfate proteoglycans in factor VIII catabolism. XVIII Congress of the International Society of Thrombosis and Haemostasis Paris, July 6-12, 2001, France. Thromb.

Haemost., Supplement, (2001), P. 206.

7. Sarafanov, A., Ananyeva, N., Shima, M., and Saenko, E. L. Role of cell surface heparan sulfate proteoglycans in fVIII catabolism. XVIII Congress of the International Society of Thrombosis and Haemostasis, Paris, France, July 6-12, 2001. Thromb.

Haemost., Supplement (2001),

Abstract

OC867.

8. Saenko, E.L. and Ananyeva, N.M. Target epitopes for factor VIII inhibitors.

XXV International Congress of the World Federation of Hemophilia, Seville, Spain, May 19-24, 2002. Hemophilia, Supplement (2002), P. 12-14.

9. Saenko, E.L. and Ananyeva, N.M. The mechanisms of inactivation of factor VIII by factor VIII inhibitors. XXV International Congress of the World Federation of Hemophilia, Seville, Spain, May 19-24, 2002. Hemophilia, Supplement (2002), P. 115 118.

10. Saenko, E.L. and Ananyeva, N. M. Role of the low-density lipoprotein-related protein in mediation of FVIII clearance. XXXV Nordic Conference on Coagulation, Reykjavik, August 10-13, 2002, Molecular Medicine. Proceedings of the XXXV Nordic Conference on Coagulation, 2002, P. 24.

11. Saenko, E. LRP mediated catabolism of factor VIII. XIX Congress of International Society of Thrombosis and Haemosytasis, Birmingham, UK, July 12-18, 2003. J. Thromb. Haemost., 2003;

Supplement 1, Abstract SY05.

12. Sarafanov, A.G., Makogonenko, Y.M., Ananyeva, N.M., Strickland, D.K., Radtke, K-P., and Saenko, E.L. Identification of residues within the A2 domain of factor VIII critical for binding to low-density lipoprotein receptor-related protein. The 45th Annual Meeting of the American Society of Hematology, San Diego, CA, December 6 9th, 2003. Blood (2003) 102: 88a.

13. Kouiavskaia, D., Ruiz, J., Strickland, D., and Saenko, E.L. Very low density lipoprotein receptor interacts with coagulation factor VIII. The 45th Annual Meeting of the American Society of Hematology, San Diego, CA, December 6-9th, 2003. Blood (2003), 102: 541a.

14. Saenko, E. and Ananyeva, N. The factor VIII protein: its life time and structure function relationship. Hemophilia 2004 World Congress, Bangkok, Thailand, October 17-21, 2004. Haemophilia, 2004;

Suppl. 10, Abstract 02 S 01.

15. Makogonenko, E.M., Sarafanov, A.G., Ananyeva, N.M., Strickland, D.K., and Saenko, E.L.Localization of binding sites for low-density lipoprotein receptor in coagulation factor VIII. 47th Annual Meeting of the American Society of Hematology, December 10-13, Atlanta, Georgia, USA. Blood (2005) 106, Abstract 1013.

16. Makogonenko, E.M., Sarafanov, A.G., Ananyeva, N.M., Radtke, K.-P., Strickland, D.K., and Saenko, E.L.B domain of coagulation factor VIII regulates exposure of its heparin-binding site. 47th Annual Meeting of the American Society of Hematology, December 10-13, 2005, Atlanta, Georgia, USA. Blood (2005) 106, Abstract 1014.

17. Sarafanov, A.G., Makogonenko, E.M., Andersen, O.M., Khrenov, A.V., Mikhailenko, I.A., Ananyeva, N.M., Strickland, D.K., and Saenko, E.L. Fragments of activated coagulation factor VIII bind to multiple sites within low-density lipoprotein receptor-related protein. 47th Annual Meeting of the American Society of Hematology, December 10-13, 2005, Atlanta, Georgia, USA. Blood (2005) 106, Abstract 1019.

18. Nogami, K., Shima, M., Nishiya, K., Saenko, E.L., Takeyama, M., Tatsumi, K., Tanaka, I., Yoshioka, A. Identification of a plasmin-interactive site within the A domain of the factor VIII heavy chain. 47th Annual Meeting of the American Society of Hematology, December 10-13, 2005, Atlanta, Georgia, USA. Blood (2005) 106, Abstract 1018.

19. Sarafanov, A., Makogonenko, E., Radtke, K-P., Pechik, I., Khrenov, A., Ananyeva, N., Strickland, D. and Saenko, E. Identification of Coagulation Factor VIII A Domain Residues Forming the Binding Epitope for Low-Density Lipoprotein Receptor Related Protein. XXth Congress of International Society of Thrombosis and Haemosytasis, 5-13 August 2005, Sydney, Australia. J. Thromb. Haemost. (2005) 3, Suppl. 1, Abstract P1840.

20. Sarafanov, A, Makogonenko, E, Andersen, O, Khrenov, A, Mikhailenko, I, Strickland, D, and Saenko, E. Identification of Regions in Low-Density Lipoprotein Receptor-Related Protein Responsible for Interaction with A2 and A1/A3-C1-C Portions of Coagulation Factor VIII. XXth Congress of International Society of Thrombosis and Haemosytasis, 5-13 August 2005, Sydney, Australia. J. Thromb.

Haemost. (2005) 3, Suppl. 1, Abstract OR248.

21. Makogonenko, Y., Sarafanov, A., Pechik, I., Andersen, O., Ananyeva, N., Radtke, K.-P., Strickland, D. and Saenko, E. The A2 domain of coagulation factor VIII shares residues critical for interaction with three members of LDL receptor superfamily LRP, VLDL and megalin receptors. XXth Congress of International Society of Thrombosis and Haemosytasis, 5-13 August 2005, Sydney, Australia. J. Thromb.

Haemost. (2005) 3, Suppl. 1, Abstract P0646.

22. Panteleev, M.A., Ananyeva, N.M., Ataullakhanov, F.I., Saenko, E.L.. Two subpopulations of activated platelets differ in their binding of the components of the intrinsic fX-activating complex. XXth Congress of the International Society on Thrombosis and Haemostasis, Sydney, Australia, August 6 - 12, 2005 J. Thromb.

Haemost. (2005), 3, Suppl. 1, Abstract P0376.

23. Kouiavskaia, D., Ruiz, J., Strickland, D. and Saenko, E. Identification of Factor VIII Binding Sites for Very Low Density Lipoprotein Receptor. XXth Congress of International Society of Thrombosis and Haemostasis, 5-13 August 2005, Sydney, Australia. J. Thromb. Haemost. (2005) 3, Suppl. 1, Abstract P1841.

24. Panteleev, M.A., Ananyeva, N.M., Greco, N.J., Ataullakhanov, F.I., Saenko, E.L.. Factor VIIIa regulates substrate delivery to the intrinsic factor X-activating complex. XXth Congress of the International Society on Thrombosis and Haemostasis, Sydney, Australia, August 6 - 12, 2005 J. Thromb. Hemost. (2005) 3, Suppl. 1, Abstract P1242.

25. Saenko, E, Ananyeva, N. Receptor-mediated Catabolism and Clearance of Factor VIII. XXth Congress of International Society of Thrombosis and Haemosytasis, 5-13 August 2005, Sydney, Australia. J. Thromb. Hemost. (2005) 3, Suppl. 1, Abstract SYM36.

26. Panteleev, M.A., Ananyeva, N.M., Ataullakhanov, F.I., Saenko, E.L. Factor VIIIa regulates substrate delivery to intrinsic factor X-activating complex. 30th FEBS Congress and 9th IUBMB Conference 'The Protein World', Budapest, Hungary, July 2-7, 2005, FEBS J. (2005) 272, Suppl. 1: 405.

27. Ananyeva, N.M., Panteleev, M.A., Greco, N.J., Ataullakhanov, F.I., Saenko, E.L. Factor VIIIa regulates substrate delivery to the intrinsic factor X-activating complex.

10th Congress of the European Hematology Association, June 25, 2005, Stockholm, Sweden, Proceedings of European J. Haematol (2005), Abstract 0312.

28. Saenko, E.L. Regulation of Factor VIII Life-Cycle by Receptors from LDL Receptor Superfamily. 36th Haemophilia Symposium, Hamburg, Germany, November 18-19, 2005. Hemophilia Therapy, Editors: W. Schramm and R. Zimmermann, Publisher: Goldener Schnitt, Sinzheim, Germany, Vol. II, 23-33.

29. Saenko, E. The future of recombinant factors. Fifth Bari Interantional Conference on Hemophilia and Allied Disorders, von Willebrand Factor and Platelet Glycoproteins, Pizzomunno, Vieste del Gargano, Italy, May 22-25, 2005. Materials of the Fifth Bari International Conference (2005), 8.

30. Saenko, E.L., Ananyeva, N.M. Life-cycle of FVIII: Implications for PK Studies in Individuals with Hemophilia. The State of the Art International Symposium on Pharmacokinetics of Factor Concentrates in Hemophilia, September 29-30, 2005, Toronto, Canada. Haemophilia (2006) Suppl. 4, 15-22.

31. Saenko, E.L. Receptor-mediated regulation of FVIII plasma level and clearance. XXth Hemophilia Forum, 16-19 March 2006, Telfs, Austria. Proceedings of the XXth Hemophilia Forum (2006), 5.



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