The Ruggeri Laboratory

Original Articles in English

1. Gagnatelli, G., Z.M. Ruggeri, P.M. Mannucci. Nervous regulation of factor VIII levels in man. Brit. J. Haemat. 20:195-207, 1971.

2. Holmberg, L., P.M. Mannucci, I. Turesson, Z.M. Ruggeri and I.M. Nilsson. Factor VIII antigen in the vessel wall in von Willebrand's disease and haemophilia A. Scand. J. Haematol. 13:33-38, 1974.

3. Mannucci, P.M., A. Capitanio, E. del Ninno, M. Colombo, F.I. Pareti and Z.M. Ruggeri. Asymptomatic liver disease in haemophiliacs. J. Clin. Pathol. 28:620-624, 1975.

4. Ruggeri, Z.M., P.M. Mannucci, S.L. Jeffcoate and G.I.C. Ingram. Immunoradiometric assay of Factor VIII related antigen, with observations in 32 patients with von Willebrand's disease. Br. J. Haematol. 33:221-232, 1976.

5. Mannucci, P.M., D. Meyer, Z.M. Ruggeri, J. Koutts, N. Ciavarella and J.M. Lavergne. Precipitating antibodies in von Willebrand's disease. Nature 262:141-142, 1976.

6. Mannucci, P.M. and Z.M. Ruggeri. Hemophilia care in Italy. Thromb. Haemost. 35:531-536, 1976.

7. Mannucci, P.M., F.I. Pareti, L. Holmberg, I.M. Nilsson and Z.M. Ruggeri. Studies on the prolonged bleeding time in von Willebrand's disease. J. Lab. Clin. Med. 88:662-671, 1976.

8. Barbui, T., F. Baudo, N. Ciavarella, G. Mariani, A. Capitanio, P.M. Mannucci, F. Pareti, Z.M. Ruggeri (Italian Working Group). Spectrum of von Willebrand's disease: a study of 100 cases. Br. J. Haematol. 35:101-112, 1977.

9. Mannucci, P.M., Z.M. Ruggeri, F.I. Pareti and A. Capitanio. 1: Deamino:8:D:Arginine vasopressin: a new pharmacological approach to the management of haemophilia and von Willebrand's disease. Lancet i:869-872, 1977.

10. Ruggeri, Z.M., P.M. Mannucci, R. Bader and T. Barbui. Factor VIII:related properties in platelets from patients with von Willebrand's disease. J. Lab. Clin. Med. 91:132-140, 1978.

11. Parekh, V.R., P.M. Mannucci and Z.M. Ruggeri. Immunological heterogeneity of hemophilia B: A multicentre study of 98 kindreds. Br. J. Haematol. 40:643-655, 1978.

12. Ruggeri, Z.M., Y.B. Gordon, N. Ardaillou, M.J. Larrieu, A. Tarantino, R. Coppola, C. Ponticelli and P.M. Mannucci. Urinary excretion of factor VIII after renal transplantation. Br. Med. J. 1:300-303, 1979.

13. Ruggeri, Z.M., N. Ciavarella, P.M. Mannucci, A. Molinari, F. Dammacco, J.M. Lavergne and D. Meyer. Familial incidence of precipitating antibodies in von Willebrand's disease. A study of four cases. J. Lab. Clin. Med. 94:60-75, 1979.

14. Ruggeri, Z.M., et al. (European Study Group). Development of Factor VIII antibody in haemophilic monozygotic twins. Scand. J. Haematol. 23:64-68, 1979.

15. Coppola, R., R. Lombardi, C. Hawkey, Z.M. Ruggeri and P.M. Mannucci. Varied immunological reactivity of Factor VIII from animal plasmas. Thromb. Res. 17:473-480, 1980.

16. Ruggeri, Z.M., F.I. Pareti, P.M. Mannucci, N. Ciavarella and T.S. Zimmerman. Heightened interaction between platelets and Factor VIII/von Willebrand Factor in a new subtype of von Willebrand's disease. N. Engl. J. Med. 302:1047-1051, 1980.

17. Ruggeri, Z.M. and T.S. Zimmerman. Variant von Willebrand's disease: characterization of two subtypes by analysis of multimeric composition of Factor VIII/von Willebrand factor in plasma and platelets. J. Clin. Invest. 65:1318-1325, 1980.

18. Mannucci, P.M., Z.M. Ruggeri, N. Ciavarella, M.D. Kazatchkine and J.F. Mowbray. Precipitating antibodies to Factor VIII/von Willebrand Factor in von Willebrand's disease: Effects on replacement therapy. Blood 57:25-31, 1981.

19. Ruggeri, Z.M. and T.S. Zimmerman. The complex multimeric composition of Factor VIII/von Willebrand factor. Blood 57:1140-1143, 1981.

20. Coppola, R., L. Guerra, Z.M. Ruggeri, A. Tarantino, P.M. Mannucci and C. Ponticelli. Factor VIII/von Willebrand Factor in glomerular nephritis. Clin. Nephrol. 16:217-222, 1981.

21. Ruggeri, Z.M., P.M. Mannucci, A.B. Federici, R. Lombardi and T.S. Zimmerman. Multimeric composition of Factor VIII/von Willebrand factor following administration of DDAVP: Implications for pathophysiology and therapy of von Willebrand's disease subtypes. Blood 59:1272-1278, 1982.

22. Lopez:Fernandez, M.F., M.H. Ginsberg, Z.M. Ruggeri, F.J. Batlle and T.S. Zimmerman. Multimeric structure of platelet Factor VIII/von Willebrand factor: The presence of larger multimers and their reassociation with thrombin stimulated platelets. Blood 60:1132-1138, 1982.

23. Ruggeri, Z.M., R. Bader and L. De Marco. Glanzmann thrombasthenia: deficient binding of von Willebrand factor to thrombin:stimulated platelets. Proc. Natl. Acad. Sci. USA 79:6038-6041, 1982.

24. Ruggeri, Z.M., I.M. Nilsson, R. Lombardi, L. Holmberg and T.S. Zimmerman. Aberrant multimeric structure of von Willebrand factor in a new variant of von Willebrand's disease (type IIC). J. Clin. Invest. 70:1124-1127, 1982.

25. Ruggeri, Z.M., R. Lombardi, L. Gatti, R. Bader, C. Valsecchi and T.S. Zimmerman. Type IIB von Willebrand's disease: Differential clearance of endogenous versus transfused large multimer von Willebrand Factor. Blood 60:1453-1456, 1982.

26. Fulcher, C.A., Z.M. Ruggeri and T.S. Zimmerman. Isoelectric focusing of human von Willebrand Factor in urea:agarose gels. Blood 61:304-310, 1983.

27. Ruggeri, Z.M., L. De Marco, L. Gatti, R. Bader and R.R. Montgomery. Platelets have more than one binding site for von Willebrand Factor. J. Clin. Invest. 72:1:12, 1983.

28. Miller, J.L., J.M. Kupinski, A. Castella and Z.M. Ruggeri. von Willebrand factor binds to platelets and induces aggregation in platelet:type but not Type IIB von Willebrand disease. J. Clin. Invest. 72:1532-1542, 1983.

29. Wahlberg, T.B., M. Blomback and Z.M. Ruggeri. Differences between heterozygous dominant and recessive von Willebrand's disease type I expressed by bleeding symptoms and combinations of factor VIII variables. Thromb. Haemost. 50:864:868, 1983.

30. McCarroll, D.R., Z.M. Ruggeri and R.R. Montgomery. The effect of DDAVP on plasma levels of von Willebrand's antigen II in normal individuals and patients with von Willebrand's disease. Blood 63:532-535, 1984.

31. McCarroll, D.R., Z.M. Ruggeri and R.R. Montgomery. Correlation between circulating levels of von Willebrand's antigen II and factor VIII/von Willebrand factor: Discrimination between Type I and Type II von Willebrand's disease. J. Lab. Clin. Med. 103:704:711, 1984.

32. Sakariassen, K.S., M. Cattaneo, A. v.d.Berg, Z.M. Ruggeri, P.M. Mannucci and J.J. Sixma. DDAVP enhances platelet adherence and platelet aggregate growth on human artery subendothelium. Blood 64:229-236, 1984.

33. Budde, U., G. Schaefer, N. Mueller, H. Egli, J. Dent, Z.M. Ruggeri and T.S. Zimmerman. Acquired von Willebrand's disease in the myeloproliferative syndrome. Blood 64:981-985, 1984.

34. Federici, A.B., J.H. Elder, L. De Marco, Z.M. Ruggeri and T.S. Zimmerman. Carbohydrate moiety of von Willebrand factor is not necessary for maintaining multimeric structure and ristocetin cofactor activity but protects from proteolytic degradation. J. Clin. Invest. 74:2049-2055, 1984.

35. Takahashi, H., M. Handa, K. Watanabe, Y. Ando, R. Nagayama, A. Hattori, A. Shibata, A.B. Federici, Z.M. Ruggeri and T.S. Zimmerman. Further characterization of platelet:type von Willebrand's disease in Japan. Blood 64:1254-1262, 1984.

36. De Marco, L., A. Girolami, S. Russell and Z.M. Ruggeri. Interaction of asialo von Willebrand factor with glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and mediates platelet aggregation. J. Clin. Invest. 75:1198:1203, 1985.

37. Saba, H.I., S.R. Saba, J. Dent, Z.M. Ruggeri and T.S. Zimmerman. Type IIB Tampa: A variant of von Willebrand disease with chronic thrombocytopenia, circulating platelet aggregates, and spontaneous platelet aggregation. Blood 66:282-286, 1985.

38. Hanna, W.T., J. Slywka, J. Dent, Z.M. Ruggeri and T.S. Zimmerman. Case report: 1:Deamino:8:D:Arginine vasopressin and cryoprecipitate in variant von Willebrand disease. Am. J. Hematol. 20:169-173, 1985.

39. Trapani Lombardo, V., E. Hodson, J.R. Roberts, T.J. Kunicki, T.S. Zimmerman and Z.M. Ruggeri. Independent modulation of von Willebrand factor and fibrinogen binding to the platelet membrane glycoprotein IIb/IIIa complex as demonstrated by monoclonal antibody. J. Clin. Invest. 76:1950-1958, 1985.

40. De Marco, L., A. Girolami, T.S. Zimmerman and Z.M. Ruggeri. Interaction of purified IIB von Willebrand factor with the platelet membrane glycoprotein Ib induces fibrinogen binding to the glycoprotein IIb/IIIa complex and initiates aggregation. Proc. Natl. Acad. Sci. USA. 82:7424-7428, 1985.

41. Ciavarella, G., N. Ciavarella, S. Antoncecchi, D. De Mattia, P. Ranieri, J. Dent, T.S. Zimmerman, and Z.M. Ruggeri. High resolution analysis of von Willebrand factor multimeric composition defines a new variant of Type I von Willebrand disease with aberrant structure but presence of all size multimers (Type IC). Blood 66:1423-1429, 1985.

42. Fujimura, Y., K. Titani, L.Z. Holland, S.R. Russell, J.R. Roberts, J.H. Elder, Z.M. Ruggeri, and T.S. Zimmerman. von Willebrand Factor: A reduced and alkylated 52/48:kDa fragment beginning at amino acid residue 449 contains the domain interacting with platelet glycoprotein Ib. J. Biol. Chem. 261:381-385, 1986.

43. Batlle J., M.F. Lopez Fernandez, J. Lasierra, Fernandez Villamor A, C. Lopez Berges, A. Lopez Borrasca, Z.M. Ruggeri, and T.S. Zimmerman. von Willebrand disease Type IIC with different abnormalities of von Willebrand factor in the same sibship. Am. J. Hematol. 21:177-188, 1986.

44. Zimmerman, T.S., J. Dent, Z.M. Ruggeri, and L.H.Nannini. Subunit composition of plasma von Willebrand factor: cleavage is present in normal individuals, increased in IIA and IIB von Willebrand disease, but minimal in variants with aberrant structure of individual oligomers (Type IIC, IID and IIE). J. Clin. Invest. 77:947-951, 1986.

45. De Marco, L., A. Girolami, T.S. Zimmerman, and Z.M. Ruggeri. von Willebrand factor interaction with the glycoprotein IIb/IIIa complex: its role in platelet function as demonstrated in patients with congenital afibrinogenemia. J. Clin. Invest. 77:1272-1277, 1986.

46. Ishibashi, T., Z.M. Ruggeri, L.A. Harker, and S.A. Burstein. Separation of human megakaryocytes by state of differentiation on continuous gradients of Percoll: size and ploidy analysis of cells identified by monoclonal antibody to glycoprotein IIb/IIIa. Blood 67:1286-1292, 1986.

47. Ruggeri, Z.M., R.A. Houghten, S.R. Russell, and T.S. Zimmerman. Inhibition of platelet function with synthetic peptides designed to be high:affinity antagonists of fibrinogen binding to platelets. Proc. Natl. Acad. Sci. USA. 83:5708-5712, 1986.

48. Mannucci, P.M., R. Lombardi, A.B. Federici, J.A. Dent, T.S. Zimmerman, and Z.M. Ruggeri. A new variant of Type II von Willebrand disease with aberrant multimeric structure of plasma but not platelet von Willebrand factor (Type IIF). Blood 68:269-274, 1986.

49. Handa, M., K. Titani, L.Z. Holland, J.R. Roberts, and Z.M. Ruggeri. The von Willebrand factor binding domain of platelet membrane glycoprotein Ib. Characterization by monoclonal antibodies and partial amino acid sequence analysis of proteolytic fragments. J. Biol. Chem. 261:12579:12585, 1986.

50. Pareti, F., Y. Fujimura, J.A. Dent, L.Z. Holland, T.S. Zimmerman and Z.M. Ruggeri. Isolation and characterization of a collagen binding domain in human von Willebrand factor. J. Biol. Chem. 261:15310-15315, 1986.

51. Budde, U., J.A. Dent, S.D. Berkowitz, Z.M. Ruggeri, and T.S. Zimmerman. Subunit composition of plasma von Willebrand factor in patients with the myeloproliferative syndrome. Blood 68:1213-1217, 1986.

52. Batlle, J., M.F. Lopez Fernandez, M. Campos, B. Justica, C. Berges, J.L. Navarro, J.M. Diaz Cremades, C.K. Kasper, J.A. Dent, Z.M. Ruggeri, and T.S. Zimmerman. The heterogeneity of Type IIA von Willebrand disease: studies with protease inhibitors. Blood 68:1207-1212, 1986.

53. Berkowitz, S.D., J. Dent, J. Roberts, Y. Fujimura, E.F. Plow, K. Titani, Z.M. Ruggeri and T.S. Zimmerman. Epitope mapping of the von Willebrand factor subunit distinguishes fragments present in normal and IIA von Willebrand disease from those generated by plasmin. J. Clin. Invest. 79:524-531, 1987.

54. Fujimura, Y., K. Titani, L.Z. Holland, J.R. Roberts, P. Kostel, Z.M. Ruggeri and T.S. Zimmerman. A heparin:binding domain of human von Willebrand factor: Characterization and localization to a tryptic fragment extending from amino acid residue Val(449) to Lys(728). J. Biol. Chem. 262:1734-1739, 1987.

55. Batlle, J., M.F. Lopez:Fernandez, A. Lopez:Borrasca, C. Lopez: Berges, J.A. Dent, S.D. Berkowitz, Z.M. Ruggeri and T.S. Zimmerman. Proteolytic degradation of von Willebrand factor after DDAVP administration in normal individuals. Blood 70:173-176, 1987.

56. De Marco, L., M. Mazzuccato, M.G. Del Ben, U. Budde, A. B. Federici, A. Girolami and Z.M. Ruggeri. Type IIB von Willebrand factor with normal sialic acid content induces platelet aggregation in the absence of ristocetin. Role of platelet activation, fibrinogen and two distinct membrane receptors. J. Clin. Invest. 80:475-482, 1987.

57. Niiya, K., E. Hodson, R. Bader, V. Byers:Ward, J.A. Koziol, E.F. Plow and Z.M. Ruggeri. Increased surface expression of the membrane glycoprotein IIb/IIIa complex induced by platelet activation. Relationship to the binding of fibrinogen and platelet aggregation. Blood 70:475-483, 1987.

58. Titani, K., K. Takio, M. Handa and Z.M. Ruggeri. Amino acid sequence of the von Willebrand factor:binding domain of platelet membrane glycoprotein Ib. Proc. Natl. Acad. Sci. USA 84:5610-5614, 1987.

59. Fujimura, Y., L.Z. Holland, Z.M. Ruggeri and T.S. Zimmerman. The von Willebrand factor domain:mediating botrocetin:induced binding to glycoprotein Ib lies between Val449 and Lys728. Blood 70:985-988, 1987.

60. Pareti, F.I., K. Niiya, J.M. McPherson and Z.M. Ruggeri. Isolation and characterization of two domains of human von Willebrand factor that interact with fibrillar collagen type I and III. J. Biol. Chem. 262:13835-13841, 1987.

61. Miller, J.L., Z.M. Ruggeri and V.A. Lyle. Unique interactions of asialo von Willebrand factor with platelets in platelet:type von Willebrand disease. Blood 70:1804-1809, 1987.

62. Ruggeri, Z.M. and T.S. Zimmerman. Classification of von Willebrand disease. J. Clin. Lab. Anal. 1:353-362, 1987.

63. Hanson, S.R., F.I. Pareti, Z.M. Ruggeri, U.M. Marzec, T.J. Kunicki, R.R. Montgomery, T.S. Zimmerman and L.A. Harker. Effects of monoclonal antibodies against the platelet glycoprotein IIb/IIIa complex on thrombosis and hemostasis in the baboon. J. Clin. Invest. 81:149-158, 1988.

64. Ngo, K.Y., V. Trifard Glotz, J.A. Koziol, D.C. Lynch, J. Gitschier, P. Ranieri, N. Ciavarella, Z.M. Ruggeri and T.S. Zimmerman. Homozygous and heterozygous deletions of the von Willebrand factor gene in patients and carriers of severe von Willebrand disease. Proc. Natl. Acad. Sci. USA 85:2753-2757, 1988.

65. Berliner, S., K. Niiya, J.R. Roberts, R.A. Houghten and Z.M. Ruggeri. Generation and characterization of peptide:specific antibodies that inhibit von Willebrand factor binding to GPIIb/IIIa without interacting with other adhesive molecules: selectivity is conferred by Pro1743 and other amino acid residues adjacent to the sequence Arg1744:Gly1745:Asp1746. J. Biol. Chem. 263:7500-7505, 1988.

66. Vicente, V., P.J. Kostel and Z.M. Ruggeri. Isolation and functional characterization of the von Willebrand factor:binding domain located between residues His1:Arg293 of the a:chain of glycoprotein Ib. J. Biol. Chem. 263:18473-18479, 1988.

67. Mohri, H., Y. Fujimura, M. Shima, A. Yoshioka, R. H. Houghten, Z.M. Ruggeri and T.S. Zimmerman. Structure of the von Willebrand factor domain interacting with glycoprotein Ib. J. Biol. Chem. 263:17901-17904, 1988.

68. Berliner, A.S., J.R. Roberts, M.L. Thorn and Z.M. Ruggeri. Generation and characterization of specific antibodies directed to the binding domain of adhesive molecules. Peptide Research 1:60-64, 1988.

69. Weiss, H.J., J. Hawiger, Z.M. Ruggeri, V.T. Turitto, P. Thiagarajan and T. Hoffmann. Fibrinogen:independent platelet adhesion and thrombus formation on subendothelium mediated by glycoprotein IIb:IIIa complex at high shear rate. J. Clin. Invest. 83:288-297, 1989.

70. Saba, H.I., Y. Fujimura, S.R. Saba, A.C. Bruce, G.A. Morelli, Z.M. Ruggeri and T.S. Zimmerman. Spontaneous platelet aggregation in Type IIB Tampa von Willebrand disease is inhibited by the 52/48:kDa fragment of normal von Willebrand factor, which contains the GPIb binding domain. Am. J. Hematol. 30:150-153, 1989.

71. Dejana, E., M.G. Lampugnani, M. Giorgi, M. Gaboli, A.B. Federici, Z.M. Ruggeri, and P.C. Machisio. Von Willebrand factor promotes endothelial cell adhesion via an Arg:Gly:Asp:dependent mechanism. J. of Cell Bio. 109:367-375, 1989.

72. Casonato, A., L. De Marco, M. Mazzucato, V. De Angelis, D. De Roia, F. Fabris, Z.M. Ruggeri and A. Girolami. A new congenital platelet abnormality characterized by spontaneous platelet aggregation, enhanced von Willebrand factor platelet interaction and the presence of all von Willebrand factor multimers in plasma. Blood 74:2028-2033, 1989.

73. Mohri, H., A. Yoshioka, T.S. Zimmerman, and Z.M. Ruggeri. Isolation of the von Willebrand factor domain interacting with platelet glycoprotein Ib, heparin, and collagen, and characterization of its three distinct functional sites. J. Biol. Chem. 264:17361-17367, 1989.

74. Cheresh, D.A., A.S. Berliner, V. Vicente and Z.M. Ruggeri. Recognition of distinct adhesive sites on fibrinogen by related integrins on platelets and endothelial cells. Cell 58:945-953, 1989.

75. Vicente, V., R.A. Houghten and Z.M. Ruggeri. Identification of a site in the alpha chain of platelet glycoprotein Ib that participates in von Willebrand factor binding. J. Biol. Chem. 265:274-280, 1990.

76. Ware, W., S.R. Russell, V. Vicente, R.E. Scharf, A. Tomer, R. McMillan and Z. M. Ruggeri. Nonsense mutation in the glycoprotein Iba coding sequence associated with Bernard:Soulier syndrome. Proc. Natl. Acad. Sci. USA 87:2026-2030, 1990.

77. Hantgan, R.R., W.L. Nichols and Z.M. Ruggeri. von Willebrand Factor competes with fibrin for occupancy of GPIIb:IIIa on thrombin:stimulated platelets. Blood 75:889-894, 1990.

78. De Marco, L., M. Mazzucato, F. Fabris, D. De Roia, P. Coser, A. Girolami, V. Vicente and Z.M. Ruggeri. Variant Bernard:Soulier syndrome type Bolzano: A cogenital bleeding disorder due to a structural and functional abnormality of the platelet glycoprotein Ib:IX complex. J. Clin. Invest. 86:25-31, 1990.

79. Budde, U., R. Schneppenheim, H. Plendl, J. Dent, Z.M. Ruggeri, and T.S. Zimmerman. Luminographic detection of von Willebrand factor multimers in agarose gels and on nitrocellulose membranes. Thromb. and Hemost. 63:312-315, 1990.

80. Nishio, K., Y. Fujimura, K. Niinomi, Y. Takashi, A. Yoshioka, H. Fukui, Y. Usami, K. Titani, Z.M. Ruggeri and T.S. Zimmerman. Enhanced botrocetin:induced type IIB von Willebrand Factor binding to platelet glycoprotein Ib initiates hyperagglutination of normal platelets. Amer. J. Hemat. 33:261-266, 1990.

81. Savage, B., U.M. Marzec, B.H. Chao, L.A. Harker, J.M. Maraganore, and Z.M. Ruggeri. Binding of the snake venom:derived proteins, applaggin and echistatin to the Arg:Gly:Asp recognition site(s) on platelet glycoprotein IIb:IIIa complex inhibits receptor function. J. Biol. Chem. 265:11766-11772, 1990.

82. Smith, J.W., Z.M. Ruggeri, T.J. Kunicki, and D.A. Cheresh. Interaction of integrins avb3 and GP IIb:IIIa with fibrinogen: Differential peptide recognition accounts for distinct binding sites. J. Biol. Chem. 265:12267-12271, 1990.

83. De Marco, L., M. Mazzucato, D. De Roia, A. Casonato, A.B. Federici, A. Girolami and Z.M. Ruggeri. Distinct abnormalities in the interaction of purified IIA and IIB von Willebrand Factor with the two platelet binding sites GP Ib:IX and GP IIb:IIIa. J. Clin. Invest. 86:785-792, 1990.

84. Dent, J.A., S.D. Berkowitz, J. Ware, C.K. Kasper and Z.M. Ruggeri. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc. Natl. Acad. Sci. USA 87:6306-6310, 1990.

85. O'Toole, T.E., J.C. Loftus, D. Xiaoping, A.A. Glass, Z.M. Ruggeri. Shattil, S.J., Plow. E.F., and Ginsberg, M.H. Affinity modulation of the aIIbb3 integrin (platelet GPIIb:IIIa) is an intrinsic property of the receptor. Cell Reg. 1:883-893, 1990.

86. Fujimura, Y., K. Titani, Y. Usami, M. Suzuki, R. Oyama, T. Matsui, H. Fukui, M. Sugimoto and Z.M. Ruggeri. Isolation and chemical characterization of two structurally and functionally distinct forms of botrocetin, the platelet coagglutinin isolated from the venom of Bothrops jararaca. Biochemistry 30:1957-1964, 1991.

87. Ware, J., J.A. Dent, H. Azuma, M. Sugimoto, P.A. Kyrle, A. Yoshioka and Z.M. Ruggeri. Identification of a point mutation in type IIB von Willebrand Disease illustrating the regulation of von Willebrand Factor affinity for the platelet GP Ib:IX receptor. Proc. Natl. Acad. Sci. USA 88:2946-2950, 1991.

88. Ikeda, Y., M. Handa, K. Kawano, T. Kamata, M. Murata, Y. Araki, H. Anbo, Y. Kawai, K. Watanabe, I. Itagaki, K. Sakai and Z.M. Ruggeri. The role of von Willebrand Factor and fibrinogen in platelet aggregation under varying shear stress. J. Clin. Invest. 87:1234-1240, 1991.

89. Savage, B., and Z.M. Ruggeri. Selective recognition of adhesive sites in surface:bound fibrinogen by GP IIb:IIIa on nonactivated platelets. J. Biol. Chem. 266:11227-11233, 1991.

90. Sugimoto, M., G. Ricca, M.E. Hrinda, A.B. Schreiber, G.H. Searfoss, E. Bottini, and Z.M. Ruggeri. Functional modulation of the isolated glycoprotein Ib:binding domain of von Willebrand factor expressed in Escherichia coli. Biochemistry 30:5202-5209, 1991.

91. Azuma, H., J.A. Dent, M. Sugimoto, Z.M. Ruggeri and J. Ware. Independent assembly and secretion of a dimeric adhesive domain of von Willebrand factor containing the glycoprotein Ib:binding site. J. Biol. Chem. 266:12342-12347,
1991.

92. Murata, M., J. Ware, and Z.M. Ruggeri. Site:directed mutagenesis of a soluble recombinant fragment of platelet glycoprotein Iba demonstrating negatively charged residues involved in von Willebrand factor binding. J. Biol. Chem. 266:15474-15480, 1991.

93. Dent, J.A., M. Galbusera, and Z.M. Ruggeri. Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J. Clin. Invest. 88:774-782, 1991.

94. Sugimoto, M., H. Mohri, R.A. McClintock, and Z.M. Ruggeri. Identification of discontinuous von Willebrand factor sequences involved in complex formation with Botrocetin: a model for the regulation of von Willebrand factor binding to platelet glycoprotein Ib. J. Biol. Chem. 266:18172-18178, 1991.

95. De Marco, L., M. Mazzucato, A. Masotti, J. W. Fenton, II, and Z.M. Ruggeri. Function of glycoprotein Iba in platelet activation induced by a:thrombin. J. Biol. Chem. 266:23776- 23783, 1991.

96. Ginsburg, D., P.L. Bockenstedt, E.A. Allen, D.A. Fox, P.A. Foster, Z.M. Ruggeri, T.S. Zimmerman, R.R. Montgomery, W.F. Bahou, T.A. Johnson, and A.Y. Yang. Fine mapping of monoclonal antibody epitopes on human von Willebrand Factor using a recombinant peptide library. Thromb. Haemost. 67:166-171, 1992.

97. Abrams, C.S., Z.M. Ruggeri, R.A. Taub, J.A. Hoxie, C. Nagaswami, J.W. Weisel, and S.J. Shattil. Anti:idiotypic antibodies against an antibody to the platelet GP IIb:IIIa complex mimic GP IIb:IIIa by recognizing fibrinogen. J. Biol. Chem. 267:2775-2785, 1992.

98. Felding:Habermann, B., Z.M. Ruggeri and D.A. Cheresh. Distinct biological consequences of integrin avb3:mediated melanoma cell adhesion to fibrinogen and its plasmic fragments. J. Biol. Chem. 267:5070-5077, 1992.

99. Murata, M., K. Furihata, F. Ishida, S.R. Russell, J. Ware and Z.M. Ruggeri. Genetic and structural characterization of an amino acid dimorphism in glycoprotein Iba involved in platelet transfusion refractoriness. Blood 79:3086-3090, 1992.

100. Savage, B., S.J. Shattil and Z.M. Ruggeri. Modulation of platelet function through adhesion receptors: A dual role for glycoprotein IIb:IIIa (Integrin aIIbb3) mediated by fibrinogen and glycoprotein Ib:von Willebrand Factor. J. Biol. Chem. 267:11300-11306, 1992.

101. Tomiyama, Y., E. Brojer, Z.M. Ruggeri, S.J. Shattil, J. Smiltneck, J. Gorski, A. Kumar, T. Kieber:Emmons, and T.J. Kunicki. A molecular model of RGD ligands: Antibody D gene segments that direct specificity for the integrin aIIbb3. J. Biol. Chem. 267:18085-18092, 1992.

102. Scharf, W.C., A. Tomer, U.M. Marzec, P.S. Teirstein, Z.M. Ruggeri and L.A. Harker. Activation of platelets in book perfusing angioplasty-damaged coronary arteries. Flow cytometric detection. Arterioscl. Thromb. 12:1475-1487, 1992.

103. Azuma, H., T. Hayashi, J.A. Dent, Z.M. Ruggeri, and J. Ware. Disulfide bond requirements for assembly of the platelet glycoprotein Ib-binding domain of von Willebrand factor. J. Biol. Chem. 268:2821-2827, 1993.

104. Alevriadou, B.R., J.L. Moake, N.A. Turner, Z.M. Ruggeri, B.J. Rolie, M.D. Phillips, A.B. Schreiber, M.E. Hrinda, and L.V. McIntire. Real-time analysis of shear-dependent thrombus formation and its blockade by inhibitors on von Willebrand factor binding to platelets. Blood 81:1263-1276, 1993.

105. Ware, J., S.R. Russell, P. Marchese and Z.M. Ruggeri. Expression of human platelet glycoprotein Iba in transgenic mice. J. Biol. Chem. 268:8376-8382, 1993.

106. Krupski, W.C., A. Bass, A.B. Kelly, Z.M. Ruggeri, L.A. Harker, and S.R. Hanson. Interruption of vascular thrombosis by bolus anti-platelet glycoprotein IIb/IIIa (GPIIb/IIIa) monoclonal antibodies in baboons. J. Vas. Surg. 17:294-304, 1993.

107. Murata, M., S.R. Russell, Z.M. Ruggeri and J. Ware. Expression of the phenotypic abnormality of platelet-type von Willebrand disease in a recombinant glycoprotein Iba fragment. J. Clin. Invest. 91:2133-2137, 1993.

108. Holmberg, L., J.A. Dent, R. Schneppenheim, U. Budde, J. Ware, and Z.M. Ruggeri. von Willebrand factor mutation enhancing interaction with platelets in patients with normal multimeric structure. J. Clin. Invest. 91:2169-2177, 1993.

109. Ikeda, Y., M. Handa, T. Kamata, K. Kawano, Y. Kawai, K. Watanabe, K. Kawakami, K. Sakai, M. Fukuyama, I. Itagaki, A. Yoshioka and Z.M. Ruggeri. Transmembrane calcium influx associated with von Willebrand factor binding to GP Ib in the initiation of shear-induced platelet aggregation. Thromb. Haemost. 69:496-502, 1993.

110. Sugimoto, M., J. Dent, R. McClintock, J. Ware and Z.M. Ruggeri. Analysis of structure-function relationships in the GP Ib-binding domain of von Willebrand factor by expression of deletion mutants. J. Biol. Chem. 268:12185-12192, 1993.

111. Prior, C.P., V. Chu, B. Cambou, J.A. Dent, B. Ebert, R. Gore, J. Holt, T. Irish, T.Lee, J. Mitschelen, R.A. McClintock, G. Searfoss. G.A. Ricca, C. Tarr, D. Weber, J.L. Ware, Z.M. Ruggeri and M. Hrinda. Optimization of a recombinant von Willebrand Factor as an Antagonist of the Platelet Glycoprotein Ib Receptor. Bio/Technology. 11:709-713, 1993.

112. Weiss, H.J., T. Hoffmann, A. Yoshioka and Z.M. Ruggeri. Evidence that the Arg1744 Gly1745 Asp1746 sequence in the GPIIb-IIIa-binding domain of von Willebrand factor is involved in platelet adhesion and thrombus formation on subendothelium. J. Lab. Clin. Med. 122:324-332, 1993.

113. Ware, J., S.R. Russell, P. Marchese, M. Murata, M. Mazzucato, L. De Marco and Z.M. Ruggeri. Point mutation in a leucine-rich repeat of platelet glycoprotein Iba resulting in the Bernard-Soulier Syndrome. J. Clin. Invest. 92:1213-1220, 1993.

114. Budde, U., R.E. Scharf, P. Franke, K. Hartmann-Budde, J. Dent and Z.M. Ruggeri. Elevated platelet count as a cause of abnormal von Willebrand factor multimer distribution in plasma. Blood 82:1749-1757, 1993.

115. Ugarova, T.P., A. Z. Budzynski, S. J. Shattil, Z.M. Ruggeri, M.H. Ginsberg, E.F. Plow. Conformational changes in fibrinogen elicited by its interaction with platelet membrane glycoprotein GPIIb-IIIa. J. Biol. Chem. 268:21080-21087, 1993.

116. Dardik, R., Z.M. Ruggeri, N. Savion, S. Gitel, U. Martinowitz, V. Chu and D. Varon. Platelet Aggregation on Extracellular Martrix: Effect of a Recombinant GPIb-Binding Fragment of von Willebrand Factor. Thromb. and Haemost. 70:522-526, 1993.

117. Benigni, A., P. Boccardo, M. Galbusera, J. Monteagudo, L. De Marco, G. Remuzzi and Z.M. Ruggeri. Reversible activation defect of the platelet glycoprotein IIb-IIIa complex in patients with uremia. Am. J. of Kidney Diseases 22:668-676, 1993.

118. Mohri, H., T.S. Zimmerman. Z.M. Ruggeri. Synthetic peptides inhibit the interaction of von Willebrand factor-platelet membrane glycoproteins. Peptides 14:125-129, 1993.

119. De Marco, L., M. Mazzucato, M., Masotti, A. and Z.M. Ruggeri. Localization and characterization of an a-thrombin-binding site on platelet glycoprotein Iba. J. Biol. Chem. 269:6478-6484, 1994.

120. Mannucci, P.M., A. Lattuada and Z.M. Ruggeri. Proteolysis of von Willebrand factor in therapeutic plasma concentrates. Blood 83:3018-3027, 1994.

121. Tomer, A., R.E. Scharf, R. McMillian, Z.M. Ruggeri, L.A. Harker. Bernard-Soulier syndrome: quantitative characterization of megakaryocytes and platelets by flow cytometric and platelet kinetic measurements. Eur J. Haematol. 52:193-200, 1994.

122. Marchese, P., M. Murata, M. Mazzucato, P. Pradella, L. De Marco, J. Ware, Z.M. Ruggeri. Identification of three tyrosine residues of GP Iba with distinct roles in von Willebrand factor and a-thrombin binding. J. Biol. Chem., 270:9571-9578, 1995.

123. Honda, S., Y. Tomiyama, A.J. Pelletier, D. Annis, Y. Honda, R. Orchekowski, Z.M. Ruggeri, T.J. Kunicki. Topography of ligand-induced binding sites (LIBS), including a novel cation-sensitive epitope (AP5) at the amino-terminus of the human integrin b3 subunit. J. Biol. Chem. 270:11947-11954, 1995.

124. Pelletier, A.J., T.J. Kunicki, Z.M. Ruggeri, V. Quaranta. The activation state of the integrin aIIbb3 affects outside:in signals leading to cell spreading and focal adhesion kinase phosphorylation. J. Biol. Chem. 270:18133-18140, 1995.

125. Pinilla, C., J. Buencamino, J.R. Appel, R.A. Houghten, J.A. Brassard, Z.M. Ruggeri. Two antipeptide monoclonal antibodies that recognize adhesive sequences in fibrinogen: Identification of antigenic determinants and unrelated sequences using synthetic combinatorial libraries. Biomedical Peptides, Proteins, and Nucleic Acids 1:199, 1995.

126. Rossi, F., R. Billetta, Z.M. Ruggeri, M. Zanetti. Engineered idiotypes: Immunochemical analysis of antigenized antibodies expressing a conformationally constrained Arg-Gly-Asp motif. Mol. Immunol. 32:341-346, 1995.

127. Ryu, G.H., J. Kim, Z.M. Ruggeri, S.H. Han, J.H. Kim, B.G. Min. Effect of shear stress on fibrinogen adsorption and its conformational change. ASAIO J. 41:M384-M388, 1995.

128. Goto, S., D.R. Salomon, Y. Ikeda, Z.M. Ruggeri. Characterization of the unique mechanism mediating the shear:dependent binding of soluble von Willebrand factor to platelets. J. Biol. Chem. 270:23352-23361, 1995.

129. Savage, B., E. Bottini, Z.M. Ruggeri. Interaction of integrin aIIbb3 with multiple fibrinogen domains during platelet adhesion. J. Biol. Chem. 270:28812-28817, 1995.

130. Savage, B., E. Saldívar, Z.M. Ruggeri. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell 84:289-297, 1996.

131. Felding-Habermann, B., R. Habermann, E. Saldívar, Z.M. Ruggeri. Role of b3 integrins in melanoma cell adhesion to activated platelets under flow. J. Biol. Chem. 271:5892-5900, 1996.

132. Miyata, S., S. Goto, A.B. Federici, J. Ware, Z.M. Ruggeri. Conformational changes in the A1 domain of von Willebrand factor modulating the interaction with platelet glycoprotein Iba. J. Biol. Chem. 271:9046-9053, 1996.

133. Schneppenheim, R., J. Brassard, S. Krey, U. Budde, T.J. Kunicki, L. Holmberg, J. Ware, Z.M. Ruggeri. Defective dimerization of von Willebrand factor subunits due to a Cys:Arg mutation in type IID von Willebrand disease. Proc. Natl. Acad. Sci. USA 93:3581-3586, 1996.

134. Stuiver, I., Z.M. Ruggeri, J.W. Smith. Divalent cations regulate the organization of integrins avb3 and avb5 on the cell surface. J. Cell. Physiol. 168:521-531, 1996.

135. Pareti, F.I., M. Cattaneo, L. Carpinelli, M.L. Zighetti, C. Bressi, P.M. Mannucci, Z.M. Ruggeri. Evaluation of the abnormal platelet function in von Willebrand disease by the blood filtration test. Thromb. and Haemost. 75:460-468, 1996.

136. Huang, S., T. Kamata, Y. Takada, Z.M. Ruggeri, G.R. Nemerow. Adenovirus interaction with distinct integrins mediates separate events in cell entry and gene delivery to hematopoietic cells. J. Virology 70:4502-4508, 1996.

137. Celikel, R. Madhusudan, K.I. Varughese, M. Shima, A. Yoshioka, J. Ware, Z.M. Ruggeri. Crystal structure of NMC:4 Fab anti:von Willebrand factor A1 domain. Blood Cells, Molecules, and Diseases 23:123-134, 1997.

138. Lanza, P., B. Felding:Habermann, Z.M. Ruggeri, M. Zanetti, R. Billetta. Selective interaction of a conformationally:constrained Arg:Gly:Asp (RGD) motif with the integrin receptor avb3 expressed on human tumor cells. Blood Cells, Molecules, and Diseases 23: 230-241, 1997.

139. Ware, J., S. Russell, Z.M. Ruggeri. Cloning of the murine glycoprotein Iba gene highlighting species:specific platelet adhesion. Blood Cells, Molecules, and Diseases 23: 292-301, 1997.

140. Federici, A.B., P.M. Mannucci, F. Stabile, M.T. Canciani, N. Di Rocco, S. Miyata, J. Ware, Z.M. Ruggeri. A type 2B von Willebrand disease mutation (Ile546:Val) associated with an unusual phenotype. Thromb. and Haemost. 78: 1132-1137, 1997.

141. Goto, S., Y. Ikeda, E. Saldívar, Z.M. Ruggeri. Distinct mechanisms of platelet aggregation as a consequent of different shearing flow conditions. J. Clin. Invest. 101: 479-486, 1998.

142. Mazzucato, M., L. De Marco, A. Masotti, P. Pradella, W.F. Bahou, Z.M. Ruggeri. Characterization of the initial a:thrombin interaction with glycoprotein Iba in relation to platelet activation. J. Biol. Chem. 273: 1880-1887, 1998.

143. Celikel, R., K.I. Varughese, Madhusudan, A. Yoshioka, J. Ware, Z.M. Ruggeri. Crystal Structure of the von Willebrand factor A1 domain in complex with the function blocking NMC-4 Fab. Nature Structural Biology 5:189-194, 1998.

144. Savage, B., F. Almus:Jacobs, Z.M. Ruggeri. Specific synergy of multiple substrate:receptor interactions in platelet thrombus formation under flow. Cell 94:657:666, 1998.

145. Kritzik, M., B. Savage, D. J. Nugent, S. Santoso, Z.M. Ruggeri, T. J. Kunicki. Nucleotide polymorphisms in the a2 gene define multiple alleles which are associated with differences in platelet a2b1 density. Blood 92:2382:2388, 1998.

146. Goto, S., H. Sakai, M. Goto, M. Ono, Y. Ikeda, S, Handa, Z.M. Ruggeri. Enhanced shear:induced platelet aggregation in acute myocardial infarction. Circulation 99:608-613, 1999.

147. Miyata, S., Z.M. Ruggeri. Distinct structural attributes regulating von Willebrand factor interaction with platelet glycoprotein Iba under flow. J. Biol. Chem. 274:6586-6593, 1999.

148. Ruggeri, Z.M., J. A. Dent, E. Saldívar. Contribution of distinct adhesive interactions to platelet aggregation in flowing blood. Blood 94:172-178, 1999.

149. Marchese, P., E. Saldívar, J. Ware, and Z.M. Ruggeri. Adhesive properties of the isolated amino terminal domain of platelet glycoprotein Iba in a flow field. Proc. Natl. Acad. Sci. USA 96:7837:7842, 1999.

150. Galbusera, M., M. Noris, C. Rossi, S. Orisio, J. Caprioli, Z. M. Ruggeri, B. Amadei, P. Ruggenenti, B. Vasile, G. Casari, G. Remuzzi. Increased fragmentation of von Willebrand factor due to abnormal cleavage of the subunit parallels disease activity in hemolytic uremic syndrome and thrombotic thrombocytopenic purpura. Blood 94:610:620, 1999.

151. Savage, B., M.H. Ginsberg, Z.M. Ruggeri. Influence of fibrillar collagen structure on the mechanisms of platelet thrombus formation under flow. Blood 94:2704-2715, 1999.

152. Ware, J., S. Russell, Z.M. Ruggeri. Generation and rescue of a murine model of platelet dysfunction - the Bernard-Soulier syndrome. Proc. Natl. Acad. Sci. USA 97:2803-2808, 2000.

153. Vasudevan, S., J.R. Roberts, R.A. McClintock, J.A. Dent, R. Celikel, J. Ware, K.I. Varughese, Z.M. Ruggeri. Modeling and functional analysis of the interaction between von Willebrand factor A1 domain and glycoprotein Iba. J. Biol. Chem. 275:12763-12768, 2000.

154. Pareti, F.I., A. Lattuada, C. Bressi, M. Zanobini, A. Sala, A. Steffan, Z. M. Ruggeri. Proteolysis of von Willebrand factor and shear stress-induced platelet aggregation in patients with aortic valve stenosis. Circulation 102:1290-1295, 2000.

155. Celikel, R., Z.M. Ruggeri, K.I. Varughese. Modulation of von Willebrand factor conformation and adhesive function by an internalized water molecule. Nature Structural Biology 7:881-884, 2000.

156. André, P., C.V. Denis, J. Ware, S. Saffaripour, R.O. Hynes, Z.M. Ruggeri, D.D. Wagner. Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins. Blood 96:3322-3328, 2000.

157. Sen, U., S. Vasudevan, G. Subbarao, R.A. McClintock, R. Celikel, Z.M. Ruggeri, K.I. Varughese. Crystal structure of the von Willebrand factor modulator botrocetin. Biochemistry 40:345-352, 2001.

158. Felding-Habermann, B., T.E. O'Toole, J.W. Smith, E. Fransvea, Z.M. Ruggeri, M.H. Ginsberg, P.E. Hughes, N. Pampori, S.S. Shattil, A. Saven, B.M. Mueller. Integrin activation controls metastasis in human breast cancer. Proc. Natl. Acad. Sci. USA 98:1853-1858, 2001.

159. Englund, G.D., R.J. Bodnar, Z. Li, Z.M. Ruggeri, X. Du. Regulation of von Willebrand factor binding to the platelet glycoprotein Ib-IX by a membrane skeleton-dependent signal. J. Biol. Chem. 276:16952-16959, 2001.

160. Schneppenheim, R., U. Budde, T. Obser, J. Brassard, S. Krey, K. Mainusch, Z.M. Ruggeri, R. Schwaab, J. Oldenburg. Expression and characterization of von Willebrand dimerization defects in different types of von Willebrand disease. Blood 97:2059-2066, 2001.

161. Soslau, G., R. Class, D.A. Morgan, C.F. Foster, S.T. Lord, P. Marchese, Z.M. Ruggeri. Unique pathway of thrombin-induced platelet aggregation mediated by glycoprotein Ib. J. Biol. Chem. 276:21173-21183, 2001.

162. Ni, H, V. Ramakrishnan, Z.M. Ruggeri, J.M. Papalia, D.R. Phillips, D. Wagner. Increased thrombogenesis and embolus formation in mice lacking glycoprotein V. Blood 98:368-373, 2001.

163. Savage, B., J.J Sixma, Z.M. Ruggeri. Functional self-association of von Willebrand factor during platelet adhesion under flow. Proc. Natl. Acad. Sci. USA 99:425-430, 2002.

164. Kasirer-Friede, A., J. Ware, L. Leng, P. Marchese, Z.M. Ruggeri, S.J. Shattil. Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin aIIbß3. J. Biol. Chem. 277:11949-56, 2002.

165. Mazzucato, M., P. Pradella, M. R. Cozzi, L. De Marco, Z. M. Ruggeri. Sequential cytoplasmic calcium signals in a two-stage platelet activation process induced by the glycoprotein Iba mechanoreceptor. Blood 100:2793-2800, 2002.

166. Sjöbring, U., U. Ringdahl, Z.M. Ruggeri. Induction of platelet thrombi by bacteria and antibodies. Blood 100:4470-4477, 2002.

167. Saldívar, E., J.N. Orje, Z.M. Ruggeri. Tensile destruction test as an estimation of partial proteolysis in fibrin clots. Am. J. Hematol. 71:119-127, 2002.

168. Gaca, J.G., A. Lesher, O. Aksoy, Z.M. Ruggeri, W. Parker W, R.D. Davis. The role of the porcine von willebrand factor: baboon platelet interactions in pulmonary xenotransplantation. Transplantation 74:1596-1603, 2002.

169. Cattaneo, M., M.L. Zighetti, R. Lombardi, C. Martinez, A. Lecchi, P.B. Conley, J. Ware, Z.M. Ruggeri. Molecular bases of defective signal transduction in the platelet P2Y12 receptor of a patient with congenital bleeding. Proc. Natl. Acad. Sci. USA 100:1978-1983, 2003.

170. Kato, K., T. Kanaji, S. Russell, T.J. Kunicki, K. Furihata, S. Kanaji, P. Marchese, A. Reininger, Z.M. Ruggeri, J. Ware. The contribution of glycoprotein VI to stable platelet adhesion and thrombus formation illustrated by targeted gene deletion. Blood, First Edition Paper, prepublished online May 8, 2003; DOI 10.1182/blood-2003-03-0717.

171. Celikel, R., R.A. McClintock, J.R. Roberts, G.L. Mendolicchio, J. Ware, K.I. Varughese, Z.M. Ruggeri. Modulation of a-thrombin function by distinct interactions with platelet glycoprotein Iba. Science 301:218-21, 2003.