Von Willebrand factor

Current Opinion in Hematology

Volumn 10, Issue 2, 2003, Pages 142-149

  Ruggeri, Zaverio M ^a  

^a SCRIPPS RESEARCH INSTITUTE   (United States)

Author keywords

[No Author keywords available]

Indexed keywords

AMINO ACID; GLYCOPROTEIN IB; POLYMER; VON WILLEBRAND FACTOR;

ACCURACY; AMINO ACID SUBSTITUTION; ARTERY DISEASE; ARTERY OCCLUSION; ARTERY THROMBOSIS; BINDING AFFINITY; BIOSYNTHESIS; BLOOD FLOW; BLOOD VESSEL INJURY; CONFORMATION; DYNAMICS; GENETIC ANALYSIS; PRIORITY JOURNAL; PROTEIN ANALYSIS; PROTEIN DOMAIN; PROTEIN FUNCTION; PROTEIN INTERACTION; PROTEIN PROTEIN INTERACTION; PROTEIN SECRETION; PROTEIN STRUCTURE; REGULATORY MECHANISM; REVIEW; STENOSIS; STRUCTURE ANALYSIS; THROMBOCYTE ACTIVATION; THROMBOCYTE FUNCTION; THROMBOGENESIS;

HUMANS; PLATELET ACTIVATION; PLATELET GLYCOPROTEIN GPIB-IX COMPLEX; PROTEIN STRUCTURE, TERTIARY; THROMBOSIS; VON WILLEBRAND FACTOR;

EID: 0037369110     PISSN: 10656251     EISSN: None     Source Type: Journal    
DOI: 10.1097/00062752-200303000-00008     Document Type: Review

References (64)

1. Ruggeri ZM: Structure and biosynthesis of von Willebrand factor. In Von Willebrand Factor and the Mechanisms of Platelet Function. Edited by Ruggeri ZM. Berlin: Springer; 1998:33-77.
2. Nogami K, Shima M, Nishiya K, et al.: A novel mechanism of factor VIII protection by von Willebrand factor from activated protein C-catalyzed inactivation. Blood 2002, 99:3993-3998.
3. Nichols WC, Cooney KA, Ginsburg D, et al.: von Willebrand disease. In Thrombosis and Hemorrhage, edn 2. Edited by Loscalzo J, Schafer AI. Baltimore: Blackwell Scientific Publications; 1998:729-755.
4. Fowler WE, Fretto LJ: Electron microscopy of von Willebrand factor. In Coagulation and Bleeding Disorders: The Role of Factor VIII and Von Willebrand Factor, edn 1. Edited by Zimmerman TS, Ruggeri ZM. New York: Marcel Dekker; 1989:181-193.
5. Siediecki CA, Lestini BJ, Kottke-Marchant K, et al.: Shear-dependent changes in the three-dimensional structure of human von Willebrand factor. Blood 1996, 88:2939-2950.
6. Novak L, Deckmyn H, Damjanovich S, et al.: Shear-dependent morphology of von Willebrand factor bound to immobilized collagen. Blood 2002, 99:2070-2076.
7. Arya M, Anvari B, Romo GM, et al.: Ultralarge multimers of von Willebrand factor form spontaneous high-strength bonds with the platelet glycoprotein Ib-IX complex: studies using optical tweezers. Blood 2002, 99:3971-3977. • Demonstrates directly the increased functional affinity of larger VWF multimers for the glycoprotein Ibα receptor.
8. Titani K, Kumar S, Takio K, et al.: Amino acid sequence of human von Willebrand factor. Biochemistry 1986, 25:3171-3184. •• A classic and monumental success of protein chemistry before the cloning era.
9. Shelton-Inloes BB, Titani K, Sadler JE: cDNA sequences for human von Willebrand factor reveal five types of repeated domains and five possible protein sequence polymorphisms. Biochemistry 1986, 25:3164-3171.
10. Mayadas TN, Wagner DD: von Willebrand factor biosynthesis and processing. Ann N Y Acad Sci 1991, 614:153-166.
11. Katsumi A, Tuley EA, Bodo I, et al.: Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor. J Biol Chem 2000, 275:25585-25594.
12. Mayadas TN, Wagner DD: Vicinal cysteines in the prosequence play a role in von Willebrand factor multimer assembly. Proc Natl Acad Sci U S A 1992, 89:3531-3535.
13. Haberichter SL, Jozwiak MA, Rosenberg JB, et al.: The von Willebrand factor propeptide (VWFpp) traffics an unrelated protein to storage. Arterioscler Thromb Vasc Biol 2002, 22:921-926. • Demonstrates the intrinsic function of the VWF propeptide for targeting proteins to storage granules.
14. Varadi K, Turecek PL, Mitterer A, et al.: Thrombin-mediated in vitro processing of pro-von Willebrand factor. Thromb Haemost 2001, 86:1149-1158.
15. van Mourik JA, Romani de Wit T, Voorberg J: Biogenesis and exocytosis of Weibel-Palade bodies. Histochem Cell Biol 2002, 117:113-122.
16. Bowie EJ, Solberg LA Jr, Fass DN, et al.: Transplantation of normal bone marrow into a pig with severe von Willebrand's disease. J Clin Invest 1986, 78:26-30.
17. Ruggeri ZM, Mannucci PM, Federici AB, et al.: Multimeric composition of factor VIII/von Willebrand factor after administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes. Blood 1982, 59:1272-1278.
18. Zimmerman TS, Dent JA, Ruggeri ZM, et al.: 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 (types IIC, IID and IIE). J Clin Invest 1986, 77:947-951. •• The first evidence that a relatively constant proportion of the VWF subunit is cleaved in all normal individuals.
19. Dent JA, Berkowitz SD, Ware J, et al.: Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci U S A 1990, 87:6306-6310. •• Identification of the peptide bond physiologically cleaved in the VWF subunit.
20. Dent JA, Galbusera M, Ruggeri ZM: Heterogeneity of plasma von Willebrand factor multimers resulting from proteolysis of the constituent subunit. J Clin Invest 1991, 88:774-782.
21. Gerritsen HE, Robles R, Lämmle B, et al.: Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001, 98:1654-1661. • One of a group of articles from different authors that in a short period established the nature of the specific VWF-cleaving protease.
22. Fujikawa K, Suzuki H, McMullen B, et al.: Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metal-loproteinase family. Blood 2001, 98:1662-1666. • One of a group of articles from different authors that in a short period established the nature of the specific VWF-cleaving protease.
23. Levy GG, Nichols WC, Lian EC, et al.: Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature 2001, 413:488-494. •• A success of positional cloning with the identification of the gene that encodes the VWF-cleaving protease.
24. Zheng X, Chung D, Takayama TK, et al.: Structure of von Willebrand factor-cleaving protease (ADAMTS13), a metalloprotease involved in thrombotic thrombocytopenic purpura. J Biol Chem 2001, 276:41059-41063. • One of a group of articles from different authors that in a short period established the nature of the specific VWF-cleaving protease.
25. Moake JL: Thrombotic microangiopathies. N Engl J Med 2002, 347:589-600. •• An excellent review from a pioneer in the field.
26. Moore JC, Hayward CP, Kelton JG: Decreased von Willebrand factor protease activity associated with thrombocytopenic disorders. Blood 2001, 98:1842-1846.
27. Remuzzi G, Galbusera M, Noris M, et al.: Von Willebrand factor cleaving protease (ADAMTS13) is deficient in recurrent and familial thrombotic thrombocytopenic purpura and hemolytic uremic syndrome. Blood 2002, 100:778-785.
28. Ajzenberg N, Denis CV, Veyradier A, et al.: Complete defect in vWF-cleaving protease activity associated with increased shear-induced platelet aggregation in thrombotic microangiopathy. Thromb Haemost 2002, 87:808-811.
29. Dong J-F, Moake JL, Nolasco L, et al.: ADAMTS-13 rapidly cleaves newly secreted ultralarge von Willebrand factor multimers on the endothelial surface under flowing conditions. Blood 2002, 100:4033-4039.
30. Mannucci PM, Canciani MT, Forza I, et al.: Changes in health and disease of the metalloprotease that cleaves von Willebrand factor. Blood 2001, 98:2730-2735.
31. Sugio Y, Okamura T, Shimoda K, et al.: Ticlopidine-associated thrombotic thrombocytopenic purpura with an IgG-type inhibitor to von Willebrand factor-cleaving protease activity. Int J Hematol 2001, 74:347-351.
32. Matsuda J, Sanaka T, Gohchi K, et al.: Occurrence of thrombotic thrombocytopenic purpura in a systemic lupus erythematosus patient with antiphospholipid antibodies in association with a decreased activity of von Willebrand factor-cleaving protease. Lupus 2002, 11:463-464.
33. Blot E, Deecaudin D, Veyradier A, et al.: Cancer-related thrombotic microangiopathy secondary to von Willebrand factor-cleaving protease deficiency. Thromb Res 2002, 106:127-130.
34. Xie L, Chesterman CN, Hogg PJ: Control of von Willebrand factor multimer size by thrombospondin-1. J Exp Med 2001, 193:1341-1349. •• A mechanism independent of proteolysis for the regulation of VWF multimer size: elegant hypothesis, but the physiologic significance remains to be proven.
35. Ruggeri ZM, Savage B: Biological functions of von Willebrand factor. In Von Willebrand Factor and the Mechanisms of Platelet Function. Edited by Ruggeri ZM. Berlin: Springer; 1998:79-109.
36. Sen U, Vasudevan S, Subbarao G, et al.: Crystal structure of the von Willebrand factor modulator botrocetin. Biochemistry 2000, 40:345-352.
37. Fukuda K, Doggett TA, Bankston LA, et al.: Structural basis of von Willebrand factor activation by the snake toxin botrocetin. Structure 2002, 10:943-950.
38. Matsui T, Hamako J, Matsushita T, et al.: Binding site on human von Willebrand factor of bitiscetin, a snake venom-derived platelet aggregation inducer. Biochemistry 2002, 41:7939-7946.
39. Savage B, Saldivar E, Ruggeri ZM: Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell 1996, 84:289-297. •• Defined the basic concepts for the role of VWF in platelet adhesion and aggregation.
40. Savage B, Almus-Jacobs F, Ruggeri ZM: Specific synergy of multiple substrate-receptor interactions in platelet thrombus formation under flow. Cell 1998, 94:657-666.
41. Celikel R, Varughese KI, Madhusudan, et al.: Crystal structure of von Willebrand factor A1 domain in complex with the function blocking NMC-4 Fab. Nat Struct Biol 1998, 5:189-194.
42. Celikel R, Ruggeri ZM, Varughese KI: von Willebrand factor conformation and adhesive function is modulated by an intemalized water molecule. Nat Struct Biol 2000, 7:881-884. •• Demonstrates the long-range propagation of conformational changes caused by a single amino acid substitution with effects on function.
43. Huizinga EG, Tsuli S, Romijn RAP, et al.: Structures of glycoprotein Ibα and its complex with von Willebrand factor A1 domain. Science 2002, 297:1176-1129. •• Provides new concepts for the structural bases of the interaction between VWF A1 domain and glycoprotein Ibα.
44. Mazzucato M, Spessotto P, Masotti A, et al.: Identification of domains responsible for von Willebrand factor type VI collagen interaction mediating platelet adhesion under high flow. J Biol Chem 1999, 274:3033-3041.
45. Wu D, Vanhoorelbeke K, Cauwenberghs N, et al.: Inhibition of the von Willebrand (VWF)-collagen interaction by an antihuman VWF monoclonal antibody results in abolition of in vivo arterial plateret thrombus formation in baboons. Blood 2002, 99:3623-3628.
46. Ribba AS, Loisel I, Lavergne JM, et al.: Ser968Thr mutation within the A3 domain of von Willebrand factor (VWF) in two related patients leads to a defective binding of VWF to collagen. Thromb Haemost 2001, 86:848-854.
47. Romijn RAP, Bouma B, Wuyster W, et al.: Identification of the collagen-binding site of the von Willebrand factor A3-domain. J Biol Chem 2001, 276:9985-9991.
48. Ruggeri ZM, Saldivar E: Platelets, hemostasis and thrombosis. In Von Willebrand Factor and the Mechanisms of Platelet Function. Edited by Ruggeri ZM Berlin: Springer; 1998:1-32.
49. Goto S, Salomon DR, Ikeda Y, et al.: Characterization of the unique mechanism mediating the shear-dependent binding of soluble von Willebrand factor to platelets. J Biol Chem 1995, 270:23352-23361.
50. Ruggeri ZM, Dent JA, Saldivar E: Contribution of distinct adhesive interactions to platelet aggregation in flowing blood. Blood 1999, 94:172-178. •• Demonstrates the physiologic role of VWF in platelet aggregation and thrombus growth.
51. 3 ligands other than VWF or fibrinogen relevant for platelet aggregation.
52. 3 ligand and provides evidence for a mechanistic link between inflammation and thrombosis.
53. Savage B, Sixma JJ, Ruggeri ZM: Functional self-association of von Willebrand factor during platelet adhesion under flow. Proc Natl Acad Sci U S A 2002, 99:425-430. •• Establishes a new concept of what constitutes the initial thrombogenic surface onto which platelets adhere.
54. Mazzucato M, Pradella P, Cozzi MR, et al.: Sequential cytoplasmic calcium signals in a two-stage platelet activation process induced by the glycoprotein Ibα mechanoreceptor. Blood 2002, 100:2793-2800.
55. 3: studies in human platelets and transfected Chinese hamster ovary cells. J Biol Chem 2000, 275:41377-41388.
56. Kasirer-Friede A, Ware J, Leng L, et al.: Lateral clustering of platelet GP Ib-IX complexes leads to up-regulation of the adhesive function of integrin alpha IIbbeta 3. J Biol Chem 2002, 277:1194g-11956.
57. 3. Blood 2002, 99:151-158.
58. 1 2) in platelet activation initiated by binding of von Willebrand factor to platelet Gp Ibα induced by conditions of high shear rate. Circulation 2002, 105:2531-2536.
59. Strony J, Beaudoin A, Brands D, et al.: Analysis of shear stress and hemodynamic factors in a model of coronary artery stenosis and thrombosis. Am J Physiol 1993, 265:H1787-H1796.
60. Mailhac A, Badimon JJ, Fallon JT, et al.: Effect of an eccentric severe stenosis on fibrin(ogen) deposition on severely damaged vessel wall in arterial thrombosis: relative contribution of fibrin(ogen) and platelets. Circulation 1994, 90:988-996.
61. Goto S, Sakai H, Goto M, et al.: Enhanced shear-induced platelet aggregation in acute myocardial infarction. Circulation 1999, 99:608-613.
62. Zareba W, Pancio G, Moss AJ, et al.: Increased level of von Willebrand factor is significantly anal independently associated with diabetes in postinfarction patients. THROMBO Investigators. Thromb Haemost 2001, 86:791-799.
63. Theilmeier G, Michiels C, Spaepen E, et al.: Endothelial von Willebrand factor recruits platelets to atherosclerosis-prone sites in response to hypercholesterolemia. Blood 2002, 99:4486-4493. • A possible mechanistic link among platelets, VWF, and altered lipid metabolism in the development of atherosclerotic lesions.
64. Methia N, Andre P, Denis CV, et al.: Localized reduction of atherosclerosis in von Willebrand factor-deficient mice. Blood 2001, 98:1424-1428. • Another aspect of the possible mechanistic link among platelets, VWF, and atherosclerosis.