The presence of active von Willebrand factor under various pathological conditions

Current Opinion in Hematology

Volumn 14, Issue 3, 2007, Pages 284-289

  Groot, Evelyn ^a   De Groot, Philip G ^a   Fijnheer, Rob ^a,b   Lenting, Peter J ^a  

^a UNIVERSITY MEDICAL CENTER UTRECHT   (Netherlands)

^b JEROEN BOSCH HOSPITAL   (Netherlands)

Author keywords

Active von Willebrand factor; Thrombocytopenia; Thrombosis

Indexed keywords

BETA2 GLYCOPROTEIN 1; VON WILLEBRAND FACTOR; VON WILLEBRAND FACTOR CLEAVING PROTEINASE;

ANTIPHOSPHOLIPID SYNDROME; AUTOIMMUNE DISEASE; BINDING KINETICS; ENZYME DEFICIENCY; HELLP SYNDROME; HEMOLYTIC ANEMIA; HUMAN; MALARIA; MICROANGIOPATHY; PLASMODIUM FALCIPARUM; PRIORITY JOURNAL; PROTEIN AGGREGATION; PROTEIN BLOOD LEVEL; PROTEIN DEGRADATION; PROTEIN FUNCTION; PROTEIN INTERACTION; PROTEIN SECRETION; REVIEW; THROMBOCYTE ADHESION; THROMBOCYTE FUNCTION; THROMBOCYTOPENIA; THROMBOSIS; THROMBOTIC THROMBOCYTOPENIC PURPURA; VON WILLEBRAND DISEASE; VON WILLEBRAND FACTOR CLEAVING PROTEINASE DEFICIENCY;

BETA 2-GLYCOPROTEIN I; DIMERIZATION; DISEASE SUSCEPTIBILITY; HUMANS; PLATELET ADHESIVENESS; VON WILLEBRAND FACTOR;

EID: 34247170871     PISSN: 10656251     EISSN: 15317048     Source Type: Journal    
DOI: 10.1097/MOH.0b013e3280dce531     Document Type: Review

References (55)

1. Ruggeri ZM. Von Willebrand factor, platelets and endothelial cell interactions. J Thromb Haemost 2003; 1:1335-1342.
2. Hulstein JJ, de Groot PG, Silence K, et al. A novel nanobody that detects the gain-of-function phenotype of von Willebrand factor in ADAMTS13 deficiency and von Willebrand disease type 2B. Blood 2005; 106:3035-3042.
3. Sadler JE. New concepts in von Willebrand disease. Annu Rev Med 2005; 56:173-191.
4. Wagner DD. Cell biology of von Willebrand factor. Annu Rev Cell Biol 1990; 6:217-246.
5. Vischer UM, Wagner DD. von Willebrand factor proteolytic processing and multimerization precede the formation of Weibel-Palade bodies. Blood 1994; 83:3536-3544.
6. Giddings JC, Brookes LR, Piovella F, Bloom AL. Immunohistological comparison of platelet factor 4 (PF4), fibronectin (Fn) and factor VIII related antigen (VIIIR:Ag) in human platelet granules. Br J Haematol 1982; 52:79-88.
7. Wagner DD, Saffaripour S, Bonfanti R, et al. Induction of specific storage organelles by von Willebrand factor propolypeptide. Cell 1991; 64:403-413.
8. André P, Denis CV, Ware J, et al. Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins. Blood 2000; 96:3322-3328.
9. Bernardo A, Ball C, Nolasco L, et al. Effects of inflammatory cytokines on the release and cleavage of the endothelial cell-derived ultralarge von Willebrand factor multimers under flow. Blood 2004; 104:100-106.
10. Padilla A, Moake JL, Bernardo A, et al. P-selectin anchors newly released ultralarge von Willebrand factor multimers to the endothelial cell surface. Blood 2004; 103:2150-2156.
11. Dong JF, 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.
12. Dong JF. Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions. J Thromb Haemost 2005; 3:1710-1716.
13. van Schooten CJ, Denis CV, Lisman T, et al. Variations in glycosylation of von Willebrand factor with O-linked sialylated T-antigen are associated with its plasma levels. Blood 2006; 7 November [Epub ahead of print].
14. Ruggeri ZM, Mannucci PM, Lombardi R, et al. Multimeric composition of factor VIII/von Willebrand factor following administration of DDAVP: implications for pathophysiology and therapy of von Willebrand's disease subtypes. Blood 1982; 59:1272-1278.
15. Sporn LA, Marder VJ, Wagner DD. Inducible secretion of large, biologically potent von Willebrand factor multimers. Cell 1986; 46:185-190.
16. Moake JL, Rudy CK, Troll JH, et al. Unusually large plasma factor VIII: von Willebrand factor multimers in chronic relapsing thrombotic thrombocytopenic purpura. N Engl J Med 1982; 307:1432-1435.
17. Tsai HM, Nagel RL, Hatcher VB, Sussman II. Multimeric composition of endothelial cell-derived von Willebrand factor. Blood 1989; 73:2074-2076.
18. Fernandez MF, Ginsberg MH, Ruggeri ZM, et al. Multimeric structure of platelet factor VIII/von Willebrand factor: the presence of larger multimers and their reassociation with thrombin-stimulated platelets. Blood 1982; 60:1132-1138.
19. 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.
20. Xie L, Chesterman CN, Hogg PJ. Control of von Willebrand factor multimer size by thrombospondin-1. J Exp Med 2001; 193:1341-1349.
21. Pimanda JE, Ganderton T, Maekawa A, et al. Role of thrombospondin-1 in control of von Willebrand factor multimer size in mice. J Biol Chem 2004; 279:21439-21448.
22. 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.
23. Furlan M, Robles R, Lamie B. Partial purification and characterization of a protease from human plasma cleaving von Willebrand factor to fragments produced by in vivo proteolysis. Blood 1996; 87:4223-4234.
24. Tsai HM. Physiologic cleavage of von Willebrand factor by a plasma protease is dependent on its conformation and requires calcium ion. Blood 1996; 87:4235-4244.
25. Gerritsen HE, Robles R, Lammle B, Furlan M. Partial amino acid sequence of purified von Willebrand factor-cleaving protease. Blood 2001; 98:1654-1661.
26. Fujikawa K, Suzuki H, McMullen B, Chung D. Purification of human von Willebrand factor-cleaving protease and its identification as a new member of the metalloproteinase family. Blood 2001; 98:1662-1666.
27. 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.
28. 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.
29. Tsai HM, Lian EC. Antibodies to von Willebrand factor-cleaving protease in acute thrombotic thrombocytopenic purpura. N Engl J Med 1998; 339:1585-1594.
30. Furlan M, Robles R, Solenthaler M, et al. Deficient activity of von Willebrand factor-cleaving protease in chronic relapsing thrombotic thrombocytopenic purpura. Blood 1997; 89:3097-3103.
31. Motto DG, Chauhan AK, Zhu G, et al. Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice. J Clin Invest 2005; 115:2752-2761.
32. Huang J, McEver R, Heuser J, Sadler J. Morphological and functional studies of ultra long von Willebrand factor (ULVWF) anchored on endothelial surface. ASH Annual Meeting Abstracts 2006; 108:1784.
33. Chauhan AK, Goerge T, Schneider SW, Wagner AD. Formation of platelet strings and microthrombi in the presence of ADAMTS13 inhibitor does not require P-selectin or beta3 integrin. J Thromb Haemost 2006; 13 December [Epub ahead of print].
34. Chauhan AK, Motto DG, Lamb CB, et al. Systemic antithrombotic effects of ADAMTS13. J Exp Med 2006; 203:767-776. This interesting paper provides evidence that ADAMTS13 is not only required for the inactivation of freshly released VWF at the endothelial surface, but also promotes thrombus dissolution in injured arterioles. The data from this paper and of Ref. [35••] demonstrate the antithrombotic properties of ADAMTS13 during thrombus formation.
35. Donadelli R, Orje JN, Capoferri C, et al. Size regulation of von Willebrand factor-mediated platelet thrombi by ADAMTS13 in flowing blood. Blood 2006; 107:1943-1950. The authors describe a novel mechanism by which VWF contributes to thrombus formation under conditions of very high shear. In addition, the size of the formed thrombi is strongly influenced by ADAMTS13, in that the presence of this protease results in smaller thrombi.
36. Huizinga EG, Tsuji S, Romijn RA, et al. Structures of glycoprotein Ibalpha and its complex with von Willebrand factor A1 domain. Science 2002; 297:1176-1179.
37. 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.
38. Ulrichts H, Udvardy M, Lenting PJ, et al. Shielding of the A1 domain by the D'D3 domains of von Willebrand factor modulates its interaction with platelet glycoprotein Ib-IX-V. J Biol Chem 2006; 281:4699-4707.
39. Nakayama T, Matsushita T, Dong Z, et al. Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb: importance of structural integrity of the regions flanked by the CYS1272-CYS1458 disulfide bond. J Biol Chem 2002; 277:22063-22072.
40. Baumgartner HR, Tschopp TB, Meyer D. Shear rate dependent inhibition of platelet adhesion and aggregation on collagenous surfaces by antibodies to human factor VIII/von Willebrand factor. Br J Haematol 1980; 44:127-139.
41. Ruggeri ZM, Orje JN, Habermann R, et al. Activation-independent platelet adhesion and aggregation under elevated shear stress. Blood 2006; 108:1903-1910. In this interesting paper, a phenomenon is described in which very high shear stress induces the formation of VWF-dependent platelet aggregates without concurrent platelet activation.
42. Sadler JE, Budde U, Eikenboom JC, et al. Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor. J Thromb Haemost 2006; 4:2103-2114.
43. Tsai HM. Current concepts in thrombotic thrombocytopenic purpura. Annu Rev Med 2006; 57:419-436.
44. Baxter JK, Weinstein L. HELLP syndrome: the state of the art. Obstet Gynecol Surv 2004; 59:838-845.
45. Rehberg JF, Briery CM, Hudson WT, et al. Thrombotic thrombocytopenic purpura masquerading as hemolysis, elevated liver enzymes, low platelets (HELLP) syndrome in late pregnancy. Obstet Gynecol 2006; 108:817-820.
46. Baron JM, Baron BW. Thrombotic thrombocytopenic purpura and its look-alikes. Clin Adv Hematol Oncol 2005; 3:868-874.
47. Hulstein JJ, Runnard Heimel PJ, Franx A, et al. Acute activation of the endothelium results in increased levels of active von Willebrand factor in hemolysis, elevated liver enzymes and low platelets (HELLP) syndrome. J Thromb Haemost 2006; 4:2569-2575. The authors show that HELLP syndrome patients are characterized by increased levels of active VWF compared with controls. These increased levels seem to originate from an exaggerated activation of the endothelium that coincides with mildly reduced ADAMTS13 activity.
48. Lattuada A, Rossi E, Calzarossa C, et al. Mild to moderate reduction of a von Willebrand factor cleaving protease (ADAMTS-13) in pregnant women with HELLP microangiopathic syndrome. Haematologica 2003; 88:1029-1034.
49. van Mourik JA, Romani de Wit T. Von Willebrand factor propeptide in vascular disorders. Thromb Haemost 2001; 86:164-171.
50. Hollestelle MJ, Donkor C, Mantey EA, et al. von Willebrand factor propeptide in malaria: evidence of acute endothelial cell activation. Br J Haematol 2006; 133:562-569.
51. Verhage DF, Telgt DS, Bousema JT, et al. Clinical outcome of experimental human malaria induced by Plasmodium falciparum-infected mosquitoes. Neth J Med 2005; 63:52-58.
52. Othman M, Labelle A, Mazzetti I, et al. Adenovirus-induced thrombocytopenia: the role of von Willebrand factor and P-selectin in mediating accelerated platelet clearance. Blood 2006; 5 December [Epub ahead of print].
53. de Groot PG, Derksen RH. Pathophysiology of the antiphospholipid syndrome. J Thromb Haemost 2005; 3:1854-1860.
54. de Laat HB, Derksen RH, Urbanus RT, et al. Beta2-glycoprotein I-dependent lupus anticoagulant highly correlates with thrombosis in the antiphospholipid syndrome. Blood 2004; 104:3598-3602.
55. Lenting P, Hulstein J, de Laat B, et al. Beta2-glycoprotein I interferes with von Willebrand factor-dependent platelet adhesion. ASH Annual Meeting Abstracts 2006; 108:415.