Insights from von Willebrand disease animal models

Cellular and Molecular Life Sciences

Volumn 56, Issue 11-12, 1999, Pages 977-990

  Denis, C V ^a   Wagner, D D ^a  

^a HARVARD MEDICAL SCHOOL   (United States)

Author keywords

Animal models; Atherosclerosis; Hemostasis; Thrombosis; von Willebrand disease; von Willebrand factor

Indexed keywords

BLOOD CLOTTING FACTOR 8; VON WILLEBRAND FACTOR;

ANIMAL MODEL; ATHEROSCLEROSIS; BLOOD CLOTTING; BONE MARROW TRANSPLANTATION; GENE MUTATION; HEMOSTASIS; HUMAN; NONHUMAN; REVIEW; THROMBOCYTE ADHESION; THROMBOSIS; VON WILLEBRAND DISEASE;

ANIMALS; ANTIGENS; ARTERIOSCLEROSIS; BLEEDING TIME; BLOOD COAGULATION; BONE MARROW TRANSPLANTATION; DISEASE MODELS, ANIMAL; DOGS; HUMANS; MICE; MICE, KNOCKOUT; PLATELET AGGREGATION; SHWARTZMAN PHENOMENON; SWINE; THROMBOSIS; VON WILLEBRAND DISEASE; VON WILLEBRAND FACTOR;

ANIMALIA; CANIS FAMILIARIS;

EID: 0033385938     PISSN: 1420682X     EISSN: None     Source Type: Journal    
DOI: 10.1007/s000180050487     Document Type: Review

References (137)

1. 1 von Willebrand E. A. (1926) Hereditär pseudohämofili. Fin. Läkäresallsk. Handl 67: 7-12
2. 2 von Willebrand E. A. (1931) Über hereditäre Pseudohämofilie. Acta. Med. Scand. 76: 521-550
3. 3 Bouma B. N., Wiegerinck Y., Sixma J. J., Van Mourik J. A. and Mochtar I. A. (1972) Immunological characterization of purified anti-haemophilic factor A (factor VIII) which corrects abnormal platelet retention in von Willebrand's disease. Nature New Biol. 236: 104 106
4. 4 Hoyer L. W. and Shainoff J. R. (1980) FVIII-related protein circulates in normal human plasma as high molecular weight multimers. Blood 55: 1056 1059
5. 5 Titani K., Kumar S., Takio K., Ericsson L. H., Wade R. D., Ashida K. et al. (1986) Amino acid sequence of human von Willebrand factor. Biochemistry 25: 3171 3184
6. 6 Bonthron D. T., Orr E. C., Mitsock L. M., Ginsburg D., Handin R. I. and Orkin S. H. (1986) Nucleotide sequence of pre-pro-von Willebrand factor cDNA. Nucleic Acids Res. 14: 7125 7127
7. 7 Shelton-Inloes B. B., Titani K. and Sadler J. E. (1986) cDNA sequences for human von Willebrand factor reveal five types of repeated domains and five possible protein sequences polymorphisms. Biochemistry 25: 3164-3171
8. 8 Verweij C. L., Diergaarde P. J., Hart M. and Pannekoek H. (1986) Full length von Willebrand factor (vWf) cDNA encodes a highly repetitive protein considerably larger than the mature vWf subunit. EMBO J. 5: 1839 1847
9. 9 Shelton-Inloes B. B., Broze G. R. J., Miletich J. P. and Sadler J. E. (1987) Evolution of human von Willebrand factor: cDNA sequence polymorphisms, repeated domains and relationship to von Willebrand antigen II. Biochem. Biophys. Res. Commun. 144: 657 665
10. 10 Mancuso D. J., Tuley E. A., Westfield L. A., Worral N. K., Shelton-Inloes B. B., Sorace J. M. et al. (1989) Structure of the gene for human von Willebrand factor. J. Biol. Chem. 264: 19514 19527
11. 11 Ewenstein B. M. (1997) von Willebrand's disease. Annu. Rev. Med. 48: 525 542
12. 12 Ruggeri Z. M. and Zimmerman T. S. (1981) The complex multimeric composition of factor VIII von Willebrand factor. Blood 57: 1140 1143
13. 13 Jaffe E. A., Hoyer L. W. and Nachman R. L. (1973) Synthesis of antihemophilic factor antigen by cultured human endothelial cells. J. Clin. Invest. 52: 2757-2764
14. 14 Nachman R., Levine R. and Jaffe E. A. (1977) Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes. J. Clin. Invest. 60: 914-921
15. 15 Wagner D. D. (1990) Cell biology of von Willebrand factor. Annu. Rev. Cell Biol. 6: 217 246
16. 16 Sporn L. A., Marder V. J. and Wagner D. D. (1986) Inducible secretion of large, biologically potent von Willebrand factor multimers. Cell 46: 185-190
17. 17 Sakariassen K. S., Bolhuis P. A. and Sixma J. J. (1979) Human blood platelets adhesion to artery subendothelium is mediated by factor VIII/von Willebrand factor bound to the subendothelium. Nature 279: 636 638
18. 18 Bolhuis P. A., Sakariassen K. S., Sander H. J., Bouma B. N. and Sixma J. J. (1981) Binding of FVIII-von Willebrand factor to human arterial subendothelium precedes increased platelet adherence and enhances platelet spreading. J. Lab. Clin. Med. 97: 568-576
19. 19 Meyer D. and Baumgartner H. R. (1983) Role of von Willebrand factor in platelet adhesion to the subendothelium. Br. J. Haematol. 54: 1 9
20. 20 Weiss H. J., Turitto V. T. and Baumgartner H. R. (1978) Effect of shear rate on platelet interaction with subendothelium in citrate and native blood. I. Shear rate-dependent decrease of adhesion in von Willebrand's disease and the Bernard-Soulier syndrome. J. Lab. Clin. Med. 92: 750-764
21. 21 Baumgartner H. R., Tschopp T. B. and Meyer D. (1980) Shear rate dependent inhibition of platelet adhesion and aggregation on collageneous surfaces by antibodies to human FVIII/von Willebrand factor. Br. J. Haematol. 44: 127-139
22. 22 Turitto V. T., Weiss H. J. and Baumgartner H. R. (1984) Platelet interaction with rabbit subendothelium in von Willebrand's disease: altered thrombus formation distinct from defective platelet adhesion. J. Clin. Invest. 74: 1730-1741
23. 23 Ikeda Y., Handa M., Kawano K., Kamata T., Murata M., Araki Y. et al. (1991) The role of von Willebrand factor and fibrinogen in platelet aggregation under varying shear stress. J. Clin. Invest. 87: 1234 1240
24. 24 Weiss H. J., Sussman I. I. and Hoyer L. W. (1977) Stabilization of factor VIII in plasma by the von Willebrand factor. J. Clin. Invest. 60: 390 404
25. 25 Brinkhous K. M., Robert L., Read M. S., Nichols T. C., Bellinger D. A. and Griggs T. R. (1991) von Willebrand factor and animal models: contributions to gene therapy, thrombotic thrombocytopenic purpura and coronary thrombosis. Mayo Clin. Proc. 66: 733 742
26. 26 Weiss H. J. (1974) Relation of von Willebrand factor to bleeding time. N. Engl. J. Med. 291: 420
27. 27 Lind S. E. (1984) Prolonged bleeding time. Am. J. Med. 77: 305 312
28. 28 Burns E. R. and Lawrence C. (1989) Bleeding time. A guide to its diagnostic and clinical utility. Arch. Pathol. Lab. Med. 113: 1219 1224
29. 29 Lamme S., Wallmark A., Holmberg L., Nilsson I. M. and Sjogren H. O. (1985) The use of monoclonal antibodies in measuring FVIII/von Willebrand factor. Scand. J. Clin. Lab. Invest. 15: 17 26
30. 30 MacFarlane D. E., Stibbe K., Kirby E. P., Zucker M. B., Grant R. A. and McPherson J. (1975) A method for assaying von Willebrand factor (ristocetin cofactor). Thromb. Diath. Haemorrh. 34: 306 308
31. 31 Weiss H. J., Rogers J. and Brand H. (1973) Defective ristocetin-induced platelet aggregation in von Willebrand's disease and its correction by factor VIII. J. Clin. Invest. 52: 2697-2707
32. 32 Forbes C. D. and Prentice C. R. M. (1973) Aggregation of human platelets by purified porcine and bovine antihaemophilic factor. Nature: New Biology 241: 149
33. 33 Altieri D., Capitanio A. M. and Mannucci P. M. (1986) von Willebrand factor contaminating porcine FVIII concentrate (Hyate: C) causes platelet aggregation. Br. J. Haematol. 63: 703 711
34. 34 Bahnak B. R., Lavergne J.-M., Ferreira V., Kerbiriou-Nabias D. and Meyer D. (1992) Comparison of the primary structure of the functional domains of human and porcine von Willebrand factor that mediates platelet adhesion. Biochem. Biophys. Res. Commun. 182: 561 568
35. 35 Bakhshi M. R., Myers J. C., Howard P. S., Soprano D. R. and Kirby E. P. (1992) Sequencing of the primary adhesion domain of bovine von Willebrand factor. Biochem. Biophys. Acta 1132: 325-328
36. 36 Sadler J. E. (1994) A revised classification of von Willebrand disease. Thromb. Haemost. 71: 520-525
37. 37 Thomas J. S. (1996) von Willebrand's disease in the dog and cat. Vet. Clin. North Am. Small Anim. Pract. 26: 1089 1110
38. 38 Kaufman R. J. (1992) Biological regulation of Factor VIII activity. Annu. Rev. Med. 43: 325 339
39. 39 Hogan A. G., Muhrer M. E. and Bogart R. (1941) A hemophilia-like disease in swine. Proc. Soc. Exptl. Biol. Med. 48: 217 219
40. 40 Mertz E. T. (1942) The anomaly of a normal Duke's and a very prolonged saline bleeding time in swine suffering from an inherited bleeding disease. Am. J. Physiol. 136: 360-362
41. 41 Muhrer M. E., Lechler E., Cornell C. N. and Kirkland J. L. (1965) Anti-hemophilic factor levels in bleeder swine following infusions of plasma and serum. Am. J. Phys. 208: 508-510
42. 42 Chan J. Y. S., Owen C. A. Jr, Bowie E. J. W., Didisheim P., Thompson J. H. Jr, Muhrer M. E. et al. (1968) von Willebrand disease 'stimulating factor' in porcine plasma. Am. J. Phys. 214: 1219-1224
43. 43 Fass D. N., Brockway W. J., Owen C.A. Jr and Bowie E. J. W. (1976) Factor VIII (Willebrand) antigen and ristocetin-Willebrand factor in pigs with von Willebrand's disease. Thromb. Res. 8: 319-327
44. 44 Brinkhous K. M. (1978) Animal models: Importance in research on hemorrhage and thrombosis. Adv. Exp. Med. Biol. 102: 123-133
45. 45 Bowie E. J. W., Owen C.A. Jr, Zollman P. E., Thompson J. H. Jr and Fass D. N. (1973) Test of hemostasis in swine: normal values and values in pigs affected with von Willebrand's disease. Am. J. Vet. Res. 34: 1405 1407
46. 46 Fass D. N., Bowie E. J. W., Owen C.A. Jr and Zollman P. E. (1979) Inheritance of porcine von Willebrand's disease: study of a kindred of over 700 pigs. Blood 53: 712-719
47. 47 Wu Q. Y., Drouet L., Carrier J. L., Rothschild C., Berard M., Rouault C. et al. (1987) Differential distribution of von Willebrand factor in endothelial cells. Comparison between normal pigs and pigs with von Willebrand disease. Arteriosclerosis 7: 47 54
48. 48 Bahou W. F., Bowie E. J. W., Fass D. N. and Ginsburg D. (1988) Molecular genetic analysis of porcine von Willebrand disease: tight linkage to the von Willebrand factor locus. Blood 72: 308 313
49. 49 Sjoberg A., Seaman W. T., Bellinger D. A., Griggs T. R., Nichols T. C. and Chowdhary B. P. (1996) FISH mapping of the porcine vWF gene to chromosome 5q21 extends synteny homology with human chromosome 12. Hereditas 124: 199 202
50. 50 Wu Q. Y., Bahnak B. R., Coulombel L., Kerbiriou-Nabias D., Drouet L., Pietu G. et al. (1988) Analysis of von Willebrand factor mRNA from the lungs of pigs with severe von Willebrand disease by using a human cDNA probe. Blood 71: 1341 1346
51. 51 Lewis J. C. and Bowie F. J. W. (1978) Ultrastructural studies of platelets of von Willebrand and normal swine. 53: 179-183
52. 52 Cramer E. M., Caen J. P., Drouet L. and Breton-Gorius J. (1986) Absence of tubular structures and immunolabeling for von Willebrand factor in the platelets x-granules from porcine von Willebrand disease. Blood 68: 774-778
53. 53 Sawada Y., Fass D. N., Katzman J. A., Bahn R. C. and Bowie E. J. W. (1986) Hemostatic plug formation in normal and von Willebrand pigs: the effect of administration of cryoprecipitate and a monoclonal antibody to von Willebrand factor. Blood 67: 1229 1239
54. 54 Reddick R. L., Griggs T. R., Lamb M. A. and Brinkhous K. M. (1982) Platelet adhesion to damaged coronary arteries: comparison in normal and von Willebrand disease swine. Proc. Natl. Acad. Sci. USA 79: 5076 5079
55. 55 Badimon L., Badimon J. J., Turrito V.T. and Fuster V. (1989) Role of von Willebrand factor in mediating platelet-vessel wall interaction at low shear rate; the importance of perfusion conditions. Blood 73: 961-967
56. 56 Bowie E. J. W., Solberg L. A. Jr, Fass D. N., Johnson C. M., Knutson G. J., Stewart M. L. et al. (1986) Transplantation of normal bone marrow into a pig with severe von Willebrand's disease. J. Clin. Invest. 78: 26 30
57. 57 Nichols T. C., Samama C. M., Bellinger D. A., Roussi J., Reddick R. L., Bonneau M. et al. (1995) Function of von Willebrand factor after crossed bone marrow transplantation between normal and von Willebrand disease pigs: effect on arterial thrombosis in chimeras. Proc. Natl. Acad. Sci. USA 92: 2455-2459
58. 58 Andre P., Brouland J. P., Roussi J., Bonneau M., Pignaud G., Bal dit Sollier C. et al. (1998) Role of plasma and platelet von Willebrand factor in arterial thrombogenesis and hemostasis in the pig. Hematology 26: 1-7
59. 59 Roussi J., Samama M., Vaiman M., Nichols T., Pignaud G., Bonneau M. et al. (1996) An experimental model for testing von Willebrand factor function: successful SLA-matched crossed bone marrow transplantations between normal and von Willebrand pigs. Exp. Hematol. 24: 585-591
60. 60 Duguid J. B. (1946) Thrombosis as a factor in the pathogenesis of coronary atherosclerosis. J. Pathol. Bacteriol. 58: 207-212
61. 61 Stemerman M. B. and Ross R. (1972) Experimental arteriosclerosis. I. Fibrous plaque formation in primates, and electron microscope study. J. Exp. Med. 136: 769 789
62. 62 Ross R., Glomset J., Kariya B. and Harker L. (1974) A platelet-dependent serum factor that stimulates the proliferation of arterial smooth muscle cells. Proc. Natl. Acad. Sci. USA 71: 1207-1210
63. 63 Harker L. A., Ross R., Slichter S. J. and Scott C. R. (1976) Homocystine-induced arteriosclerosis: the role of endothelial cell injury and platelet response to its genesis. J. Clin. Invest. 58: 731 741
64. 64 Fuster V., Badimon L., Badimon J. J., Ip J. H. and Chesebro J. H. (1991) The porcine model for the understanding of thrombogenesis and atherogenesis. Mayo Clin. Proc. 66: 818-831
65. 65 Fuster V., Bowie E. J. W., Lewis J. C., Fass D. N. and Owen C. A. Jr (1978) Resistance to atherosclerosis in pigs with von Willebrand's disease. Spontaneous and high cholesterol diet-induced arteriosclerosis. J. Clin. Invest. 61: 722-730
66. 66 Fuster V. and Bowie E. J. W. (1978) The von Willebrand pig as a model for atherosclerosis research. Thromb. Haemost. 39: 322-327
67. 67 Badimon L., Steele P., Badimon J. J., Bowie E. J. W. and Fuster V. (1985) Aortic atherosclerosis in pigs with heterozygous von Willebrand disease. Comparison with homozygous von Willebrand and normal pigs. Arteriosclerosis 5: 366 370
68. 68 Griggs T. R., Bauman R. W., Reddick R. L., Read M. S., Koch G. G. and Lamb M. A. (1986) Development of coronary atherosclerosis in swine with severe hypercholesterolemia. Lack of influence of von Willebrand factor or acute intimal injury. Arteriosclerosis 6: 155 165
69. 69 Nichols T. C., Bellinger D. A., Davis K. E., Koch G. G., Reddick R. L., Read M. S. et al. (1992) Porcine von Willebrand disease and atherosclerosis. Influence of polymorphism in apolipoprotein B100 genotype. Am. J. Pathol. 140: 403-415
70. 70 Federici A. B., Mannucci P. M., Fogato E., Ghidoni P. and Matturri L. (1993) Autopsy findings in three patients with von Willebrand disease type IIB and type III: presence of atherosclerotic lesions without occlusive arterial thrombi. Thromb. Haemost. 70: 758 761
71. 71 Reddick R. L., Read M. S., Brinkhous K. M., Bellinger D., Nichols T. and Griggs T. R. (1990) Coronary atherosclerosis in the pig. Induced plaque injury and platelet response. Arteriosclerosis 10: 541 550
72. 72 Nichols T. C., Bellinger D. A., Tate D. A., Reddick R. L., Read M. S., Koch G. G. et al. (1990) von Willebrand factor and occlusive arterial thrombosis. A study in normal and von Willebrand's disease pigs with diet-induced hypercholesterolemia and atherosclerosis. Arteriosclerosis 10: 449 461
73. 73 Nichols T. C., Bellinger D. A., Reddick R. L., Read M. S., Koch G. G. and Brinkhous K. M. et al. (1991) Role of von Willebrand factor in arterial thrombosis. Studies in normal and von Willebrand disease pigs. Circulation 83 (Suppl. IV): IV 56-IV 64
74. 74 Nichols T. C., Bellinger D. A., Reddick R. L., Koch G. G., Sigman J. L., Erickson G. et al. (1998) von Willebrand factor does not influence atherogenesis in arteries subjected to altered shear stress. Arterioscler. Thromb. Vasc. Biol. 18: 323-330
75. 75 Bowie E. J. W., Fass D. N. and Owen C.A. Jr (1980) Hemostatic effect of transfused Willebrand factor in porcine von Willebrand's disease. Similarities with human disease. Haemostasis 9: 352-365
76. 76 Griggs T. R., Webster W. P., Cooper H. A., Wagner R. H. and Brinkhous K. M. (1974) von Willebrand factor: gene dosage relationship and transfusion response in bleeder swine. A new bioassay. Proc. Natl. Acad. Sci. USA 71: 2087 2090
77. 77 Cornu P., Larrieu M. J., Caen J. and Bernard J. (1963) Transfusion studies in von Willebrand's disease: effect on bleeding time and FVIII. Br. J. Haemat. 9: 189-202
78. 78 Perkins H. A. (1967) Correction of the hemostatic defects in von Willebrand's disease. Blood 30: 375-380
79. 79 Roussi J., Turecek P. L., André P., Bonneau M., Pignaud G., Bal dit Sollier C. et al. (1998) Effects of human recombinant, plasma-derived and porcine von Willebrand factor in pigs with severe von Willebrand disease. Blood Coag. Fibrinol. 9: 361-372
80. 80 Dodds W. J. (1970) Canine von Willebrand's disease. J. Lab. Clin. Med. 76: 713-721
81. 81 Johnson G. S., Turrentine M. A. and Kraus K. H. (1988) Canine von Willebrand's disease. A heterogeneous group of bleeding disorders. Hemostasis 18: 195-229
82. 82 Mannucci P. M. (1995) Platelet von Willebrand factor in inherited and acquired bleeding disorders. Proc. Natl. Acad. Sci. USA 92: 2428-2432
83. 83 McCaroll D. R., Waters D. C, Steidley K. R., Clift R. and McDonald T. P. (1988) Canine platelet von Willebrand factor: quantification and multimeric analysis. Exp. Hematol. 16: 929 937
84. 84 Jergens A.E., Turrentine M. A., Kraus K. H. and Johnson G. S. (1987) Buccal mucosa bleeding times of healthy dogs and of dogs in various pathologic states, including thrombocytopenia, uremia and von Willebrand's disease. Am. J. Vet. Res. 48: 1337 1342
85. 85 Brooks M. and Catalfamo J. (1993) Buccal mucosa bleeding time is prolonged in canine models of primary hemostatic disorders. Thromb. Haemost. 70: 777-780
86. 86 Stokol T., Parry B. W. and Mansell P. D. (1995) Factor VIII activity in canine von Willebrand disease. Vet. Clin. Pathol. 24: 81 90
87. 87 Dodds W.J. (1984) von Willebrand's disease in dogs. Mod. Vet. Pract. 65: 681-686
88. 88 Brooks M. (1992) Management of canine von Willebrand's disease. Probl. Vet. Med. 4: 636-646
89. 89 Dodds W. J. (1975) Further studies of canine von Willebrand's disease. Blood 45: 221-230
90. 90 Meinkoth J. H. and Meyers K. M. (1995) Measurement of von Willebrand factor-specific mRNA and release and storage of von Willebrand factor from endothelial cells of dogs with type-I von Willebrand's disease. Am. J. Vet. Res. 56: 1577-1585
91. 91 Ewenstein B. M., Inbal A, Pober J. S. and Handin R. I. (1990) Molecular studies of von Willebrand disease: reduced von Willebrand factor biosynthesis, storage and release in endothelial cells derived from patients with type I von Willebrand disease. Blood 75: 1466 1472
92. 92 Johnson G. S., Turrentine M. A. and Dodds W. J. (1987) Type II von Willebrand's disease in German shorthair pointers. Vet. Clin. Pathol. 16: 7
93. 93 Rosborough T. K., Johnson G. S., Benson R. E., Swaim W. R. and Dodds W. J. (1980) Measurement of canine von Willebrand factor using ristocetin and polybrene: diagnosis of canine von Willebrand's disease. J. Lab. Clin. Med. 96: 47-56
94. 94 Schlink G. T. and Johnson G. S. (1983) A sensitive autora-diographic procedure for FVIII-related antigen in canine plasma. Vet. Clin. Pathol. 12: 21-27
95. 95 Brinkhous K. M., Read M. S., Reddick R. L. and Griggs T. R. (1981) Pathophysiology of platelet-aggregating von Willebrand factor: applications of the venom coagglutinin vWf assay. Ann. N.Y. Acad. Sci. 370: 191-204
96. 96 Benson R. E., Johnson G. S. and Dodds W. J. (1981) Binding of low-molecular-weight canine FVIII coagulant from von Willebrand plasma to canine FVIII-related antigen. Br. J. Haematol. 49: 541 550
97. 97 Dodds W. J. (1984) Bleeding diseases of small animals. Vet. Ref. Lab. Newsletter 8: 1 4
98. 98 Johnson G. S., Lees G. E., Benson R. E., Rosborough T. K. and Dodds W. J. (1980) A bleeding disorder (von Willebrand's disease) in a Chesapeake Bay Retriever. J. Am. Vet. Med. Assoc. 176: 1261-1263
99. 99 Brooks M., Raymond S. and Catalfamo J. (1996) Severe, recessive von Willebrand's disease in Dutch Kooiker dogs. J. Am. Vet. Med. Assoc. 209: 926-929
100. 100 Slappendel R. J., Beijer E. G. and van Leeuwen M. (1998) Type III von Willebrand's disease in Dutch Kooiker dogs. Vet. Q. 20: 93-97
101. 101 Raymond S. L., Jones D. W., Brooks M. B. and Dodds W. J. (1990) Clinical and laboratory features of a severe form of von Willebrand disease in Shetland Sheepdogs. J. Am. Vet. Med. Assoc. 10: 1342 1346
102. 102 Rieger M., Schwarz H. P., Turecek P. L., Dorner F., van Mourik J. A. and Mannhalter C. (1998) Identification of mutations in the canine von Willebrand factor gene associated with type III von Willebrand disease. Thromb. Haemost. 80: 332-337
103. 103 Avgeris S., Lothrop C. D. and McDonald T. P. (1990) Plasma von Willebrand factor concentration and thyroid function in dogs. J. Am. Vet. Med. Assoc. 196: 921-924
104. 104 Dalton D. G., Dewar R. G., Savidge G. F., Kernoff P. B., Matthews K. B., Greaves M. et al. (1987) Hypothyroidism as a cause of acquired von Willebrand's disease. Lancet 1: 1007 1009
105. 105 Rao K. P., Kizer J., Jones T. J., Anunciado A., Pepkowitz S. H. and Lazarchick J. (1988) Acquired von Willebrand syndrome associated with an extra nodal pulmonary lymphoma. Arch. Pathol. Lab. Med. 112: 47 50
106. 106 van Genderen P. J. J., Vink T., Michiels J. J., van't Veer M. B., Sixma J. J. and van Vliet H. H. (1994) Acquired von Willebrand disease caused by an antibody selectively inhibiting the binding of von Willebrand factor to collagen. Blood 84: 3378-3384
107. 107 Nichols T. C., Bellinger D. A., Reddick R. L., Smith S. V., Koch G. G., Davis K. et al. (1993) The roles of von Willebrand factor and factor VIII in arterial thrombosis: studies in canine von Willebrand disease and hemophilia. Blood 81: 2644 2651
108. 108 Stokol T. (1997) The roles of von Willebrand factor and factor VIII in arterial thrombosis: studies in canine von Willebrand disease and hemophilia. Res. Vet. Sci. 63: 23-27
109. 109 Turecek P. L., Gritsch H., Pichler L., Auer W., Fischer B., Mitterer A. et al. (1997) In vivo characterization of recombinant von Willebrand factor in dogs with von Willebrand disease. Blood 90: 3555 3567
110. 110 Mannucci P. M. (1997) Desmopressin (DDAVP) in the treatment of bleeding disorders: the first 20 years. Blood 90: 2515-2521
111. 111 Giger U. and Dodds W. J. (1989) Effect of desmopressin in normal dogs and dogs with von Willebrand's disease. Vet. Clin. Pathol. 18: 39 42
112. 112 Meyers K. M., Wardrop K. J. and Meinkoth J. (1992) Canine von Willebrand's disease: pathobiology, diagnosis and short-term treatment. Compend. Contin. Educ. Pract. Vet. 14: 13 21
113. 113 Meyers K. M., Wardrop K. J., Dodds W. J. and Brassard J. (1990) Effect of exercise, DDAVP and epinephrine on the factor VIIi:C/von Willebrand factor complex in normal dogs and von Willebrand factor deficient Doberman Pinscher dogs. Thromb. Res. 57: 97 108
114. 114 Sweeney J. D., Novak E. K., Reddington M., Takeuchi K. H. and Swank R. T. (1990) The RIIIS/J inbred mouse strain as a model for von Willebrand disease. Blood 76: 2258-2265
115. 115 Denis C., Methia N., Frenette P. S., Rayburn H., Ullman-Cullere M., Hynes R. O. et al. (1998) A mouse model of severe von Willebrand disease: defects in hemostasis and thrombosis. Proc. Natl. Acad. Sci. USA 95: 9524 9529
116. 116 Nichols W. C., Cooney K., Mohlke K. L., Ballew J. D., Yang A., Bruck M. E. et al. (1994) von Willebrand disease in the RIIIS/J mouse is caused by a defect outside of the von Willebrand factor gene. Blood 83: 3225 3231
117. 117 Mohlke K. A., Purkayastha A. A., Westrick R. J., Smith P. L., Petryniak B., Lowe J. B. et al. (1999) Mvwf, a dominant modifier of murine von Willebrand factor, results from altered lineage-specific expression of a glycosyltransferase. Cell 96: 111 120
118. 118 Gill J. C., Endress-Brooks J., Bauer P. J., Marks W. J. and Montgomery R. R. (1987) The effect of ABO blood group on the diagnosis of von Willebrand disease. Blood 79: 1691-1695
119. 119 Shwartzman G. (1928) A new phenomenom of local skin reactivity to B. typhosis culture filtrate. Proc. Soc. Exp. Biol. Med. 25: 560
120. 120 Rothstein J. L. and Schreiber H. (1988) Synergy between tumor necrosis factor and bacterial products causes hemorrhagic necrosis and lethal shock in normal mice. Proc. Natl. Acad. Sci. USA 85: 607 611
121. 121 Subramaniam M., Frenette P. S., Saffaripour S., Johnson R. C., Hynes R. O. and Wagner D. D. (1996) Defects in hemostasis in P-selectin-deficient mice. Blood 87: 1238 1242
122. 122 Brozna J. P. (1990) Shwartzman reaction. Semin. Thromb. Hemost. 16: 326-332
123. 123 Denis C., Williams J. A., Lu X., Meyer D. and Baruch D. (1993) Solid-phase von Willebrand factor contains a conformationally active RGD motif that mediates endothelial cell adhesion through the xvβ3 receptor. Blood 82: 3622-3630
124. 124 Kurz K. D., Main B. W. and Sandusky G. E. (1990) Rat model of arterial thrombosis induced by ferric chloride. Thromb. Res. 60: 269 280
125. 125 Benson R. E. and Dodds W. J. (1977) Autosomal factor VIII deficiency in rabbits: size variations of rabbit factor VIII. Thromb. Haemost. 38: 380
126. 126 Dodds W. J. (1981) Second international registry of animal models of thrombosis and hemorrhagic diseases. ILAR news 24: 1 50
127. 127 French T. W., Fox L. E., Randolph J. F. and Dodds W. J. (1987) A bleeding disorder (von Willebrand's disease) in a Himalayan cat. J. Am. Vet. Med. Assoc. 190: 437 439
128. 128 Mannucci P., Moia M., Rebulla P., Altieri D., Monteagudo J. and Castillo R. (1987) Correction of the bleeding time in treated patients with severe von Willebrand's disease is not solely dependent on the normal multimeric structure of plasma von Willebrand factor. Am. J. Hematol. 25: 55-65
129. 129 Hoyer L. W. (1981) The factor VIII complex: structure and function. Blood 58: 1-13
130. 130 Lollar P., Hills-Eubanks D. C. and Parker C. G. (1988) Association of the factor VIII light chain with von Willebrand factor. J. Biol. Chem. 263: 10451 10455
131. 131 Nishino M., Girma J. P., Rothschild C., Fressinaud E. and Meyer D. (1989) New variant of von Willebrand disease with defective binding to factor VIII. Blood 74: 1591 1599
132. 132 Wise R. J., Eweinstein B. M., Gorlin J., Narins S. C., Jesson M. and Handin R. I. (1993) Autosomal recessive transmission of hemophilia A due to a von Willebrand factor mutation. Hum. Genet. 91: 367 372
133. 133 Kaufman R. J., Dorner A. J. and Fass D. N. (1999) von Willebrand factor elevates plasma factor VIII without induction of factor VIII messenger RNA in the liver. Blood 93: 193 197
134. 134 Wion K. L., Kelly D., Summerfield J. A., Tuddenham E. G. D. and Lawn R. M. (1985) Distribution of factor VIII mRNA and antigen in human liver and other tissues. Nature 317: 726 729
135. 135 Visher U. M., Ingerslev J., Wollheim C. B., Mestries J. C., Tsakiris D. A., Haefeli W. E. et al. (1997) Acute von Willebrand factor secretion from the endothelium in vivo: assessment through plasma propeptide (vWf:AgII) levels. Thromb. Hemost. 77: 387-393
136. 136 Wagner D. D., Saffaripour S., Bonfanti R., Sadler J. E., Cramer E. M., Chapman B. et al. (1991) Induction of specific storage organelles by von Willebrand factor propolypeptide. Cell 64: 403 413
137. 137 Mayadas T. N., Johnson R. C., Rayburn H., Hynes R. O. and Wagner D. D. (1993) Leukocyte rolling and extravasation are severely compromised in P-selectin-deficient mice. Cell 74: 541 554