Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00035024.xml
Thromb Haemost 1993; 70(01): 099-104
DOI: 10.1055/s-0038-1646168
DOI: 10.1055/s-0038-1646168
State-of-the-Art Lecture
von Willebrand Factor
von Willebrand Factor: Structure and Function
Further Information
Publication History
Publication Date:
03 July 2018 (online)
-
References
- 1 Ruggeri ZM, Ware J. The structure and function of von Willebrand factor. Thromb Haemoslas 1992; 67: 594-9
- 2 Ginsburg D, Sadler JE. von Willebrand disease: a data base of point mutations, insertions and deletions. Thromb Haemostas 1993; 69: 177-84
- 3 Titani K, Kumar S, Takio K, Ericsson LH, Wade RD, Ashida K, Walsh KA, Chopek MW, Sadler JE, Fujikawa K. Amino acid sequence of human von Willebrand Factor. Biochemistry 1986; 25: 3171-84
- 4 Colombatti A, Bonaldo P. The superfamily of proteins with von Willebrand factor type A-like domains: One theme common to components of extracellular matrix, hemostasis, cellular adhesion, and defense mechanisms. Blood 1991; 77: 2305-15
- 5 Wagner DD. Cell biology of von Willebrand factor. Ann Rev Cell Biol 1990; 6: 217-46
- 6 Chopek MW, Girma JP, Fujikawa K, Davie EW, Titani K. Human von Willebrand factor: a multivalent protein composed of identical subunits. Biochemistry 1986; 25: 3146-55
- 7 Dent JA, Berkowilz SD, Ware J, Kasper CK, Ruggeri ZM. Identification of a cleavage site directing the immunochemical detection of molecular abnormalities in type IIA von Willebrand factor. Proc Natl Acad Sci USA 1990; 87: 6306-10
- 8 Koedam JA, Meijers JCM, Sixma JJ, Bouma BN. Inactivation of human Factor VIII by activated protein C. Cofactor activity of protein S and protective effect of von Willebrand Factor. J Clin Invesl 1988; 82: 1236-43
- 9 Koedam JA, Hamer RJ, Beeser-Visser NH, Bouma BN, Sixma JJ. The effect of von Willebrand Factor on activation of Factor VIII by Factor Xa. Eur J Biochem 1990; 189: 229-34
- 10 Kaufman RJ, Wasley LC, Dorner AJ. Synthesis, processing, and secretion of recombinant human Factor VIII expressed in mammalian cells. J Biol Chem 1988; 263: 6352-62
- 11 Nishino M, Girma JP, Rothschild C, Fressinaud E, Meyer D. New variant of von Willebrand disease with defective binding to Factor VIII. Blood 1989; 74: 1591-9
- 12 Weiss HJ, Turitto VT, Baumgartner HR. Effect of shear rate on platelet interaction with subendothelium in citrated and native blood. I. Shear rate-dependent decrease of adhesion in von Willebrand's disease and the Bernard-Soulier syndrome. J Lab Clin Med 1978; 92: 750-64
- 13 Weiss HJ, Hawiger J, Ruggeri ZM, Turitto VT, Thiagarajan P, Hoffmann T. Fibrinogen-independent platelet adhesion and thrombus formation on subendothelium mediated by glycoprotein IIb-IIIa complex at high shear rate. J Clin Invesl 1989; 83: 288-97
- 14 De Groot PG, Ottenhof-Rovers M, van Mourik JA, Sixma JJ. Evidence that the primary binding site of von Willebrand factor that mediates platelet adhesion on subendothelium is not collagen. J Clin Invest 1988; 82: 65-73
- 15 Denis C, Baruch D, Kielty CM, Ajzenberg N, Christophe O, Meyer D. Localization of von Willebrand factor binding domains to endothelial extracellular matrix and to type VI collagen. Arterioscler Thromb. 1993 13. in press
- 16 Rand JH, Nayana DP, Schwartz E, Sheng-Li Z, Potter BJ. 150-kD von Willebrand Factor binding protein extracted from human vascular subendothelium is type VI collagen. J Clin Invest 1991; 88: 253-9
- 17 Sakariassen KS, Bolhuis PA, Sixma JJ. Human blood platelet adhesion to artery subendothelium is mediated by Factor VIII-von Willebrand Factor bound to the subendothelium. Nature 1979; 279: 636-8
- 18 Fressinaud E, Baruch D, Girma JP, Sakariassen KS, Baumgartner HR, Meyer D. von Willebrand factor-mediated platelet adhesion to collagen involves platelet membrane glycoprotein IIb/IIIa as well as glycoprotein Ib. J Lab Clin Med 1988; 112: 58-67
- 19 Scott JP, Montgomery RR, Retzinger GS. Dimeric ristocetin flocculates proteins, binds lo platelets, and mediates von Willebrand factor-dependent agglutination of platelets. J Biol Chem 1991; 266: 8149-55
- 20 Andrews RK, Booth WJ, Gorman JJ, Castaldi PA, Berndt MC. Purification of botrocetin front Bothrops jararaca venom. Analysis of the botrocetin-mediated interaction between von Willebrand Factor and the human plalelet membrane glycoprotein Ib-IX complex. Biochemistry 1989; 28: 8317-26
- 21 Moake JL, Turner NA, Stathopoulos NA, Nolasco LH, Heliums JD. Involvement of large plasma von Willebrand Factor (vWF) multimers and unusually large vWF forms derived from endothelial cells in shear stress-induced platelet aggregation. J Clin Invest 1986; 78: 1456-61
- 22 Ikeda Y, Handa M, Kawano K, Kamata T, Murata M, Araki Y, Anbo H, Kawai Y, Watanabe K, Itagaki I, Sakai K, Ruggeri ZM. The role of von Willebrand Factor and fibrinogen in platelet aggregation under varying shear stress. J Clin Invest 1991; 87: 1234-40
- 23 Chow TW, Heliums JD, Moake JL, Kroll MH. Shear stress-induced von Willebrand factor binding to platelet glycoprotein Ib initiates calcium influx associated with aggregation. Blood 1992; 80: 113-20
- 24 Savage B, Shattil SJ, Ruggeri ZM. Modulation of platelet function through adhesion receptors - A dual role for glycoprotein IIb-IIIa (inlegrin-alpha(IIb)beta(3) mediated by fibrinogen and glycoprotein Ib-von Willebrand factor. J Biol Chem 1992; 267: 11300-6
- 25 Weiss HJ, Turitto VT, Baumgartner HR. Platelet adhesion and thrombus formation on subendothelium in platelets deficient in glycoproteins IIb-IIIa. Ib and storage granules. Blood 1986; 67: 322-30
- 26 De Marco L, Girolami A, Zimmerman TS, Ruggeri ZM. 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 1986; 77: 1272-7
- 27 Turitto VT, Weiss HJ, Baumgartner HR. Platelet interaction with rabbit subendothelium in von Willebrand's disease; altered thrombus formation distinct from defective platelet adhesion. J Clin Invest 1984; 74: 1730-41
- 28 Fressinaud E, Sakariassen KS, Rothschild C, Baumgartner HR, Meyer D. Shear rate dependent impairment of thrombus growth on collagen in non-anticoagulated blood from patients with von Willebrand disease and hemophilia A. Blood 1992; 80: 988-94
- 29 Houdijk WPM, de Groot PG, Nievelstein PFEM, Sakariassen KS, Sixma JJ. Subendothelial proteins and platelet adhesion, von Willebrand factor and fibronectin, not thrombospondin, are involved in platelet adhesion to extracellular matrix of human vascular endothelial cells. Arteriosclerosis 1986; 6: 24-33
- 30 Baruch D, Denis C, Marteaux C, Schoevaert D, Coulombel L, Meyer D. Role of von Willebrand factor associated lo extracellular matrices in platelet adhesion. Blood 1991; 77: 519-27
- 31 Stel HV, Sakariassen KS, De Groot PG, van Mourik JA, Sixma JJ. von Willebrand factor in the vessel wall mediates platelet adhesion. Blood 1985; 65: 85-90
- 32 Fressinaud E, Baruch D, Rothschild C, Baumgartner HR, Meyer D. Platelet von Willebrand factor: evidence for its involvement in platelet adhesion to collagen. Blood 1987; 70: 1214-17
- 33 Moake JL, Turner NA, Stathopoulos NA, Nolasco L, Heliums JD. Shear induced platelet aggregation can be mediated by vWF released from platelets, as well as by exogenous large or unusually large vWF multimers, requires adenosine diphosphate and is resistant lo aspirin. Blood 1988; 71: 1366-74
- 34 Bowie EJW, Solberg LA, Fass DN, Johnson CM, Knutson GJ, Stewart ML, Zoeklein LJ. Transplantation of normal bone marrow into a pig with severe von Willebrand's disease. J Clin Invest 1986; 78: 26-30
- 35 Marti T, Rosselet SJ, Titani K, Walsh KA. Identification of disulfide-bridged substructures within human von Willebrand Factor. Biochemistry 1987; 26: 8099-109
- 36 Andrews RK, Gorman JJ, Booth WJ, Corino GL, Castaldi PA, Berndt MC. Cross-linking of a monomeric 39/34-kDa dispase fragment of von Willebrand Factor (Leu480/Val481-Gly718) to the N-terminal region of the α-chain of membrane glycoprotein Ib on intact platelets with bis(sulfosuccinimidyl) suberate. Biochemistry 1989; 28: 8326-36
- 37 Wise RJ, Dorner AJ, Krane M, Pittman DD, Kaufman RJ. The role of von Willebrand Factor multimers and propeptide cleavage in binding and stabilization of Factor VIII. J Biol Chem 1991; 266: 21948-55
- 38 Foster PA, Fulcher CA, Marti T, Titani K, Zimmerman TS. A major Factor VIII binding domain resides within the amino-terminal 272 amino acid residues of von Willebrand Factor. J Biol Chem 1987; 262: 8443-6
- 39 Takahashi Y, Kalafatis M, Girma JP, Sewerin K, Andersson LO, Meyer D. Localization of a Factor VIII binding domain on a 34 kilodalton fragment of the N-terminal portion of von Willebrand Factor. Blood 1987; 70: 1679-82
- 40 Layet S, Girma JP, Obert B, Peynaud-Debayle E, Bihoreau N, Meyer D. Evidence that a secondary binding and protecting site for Factor VIII on von Willebrand Factor is highly unlikely. Biochem J 1992; 282: 129-37
- 41 Piétu G, Ribba AS, Meulien P, Meyer D. Localization within the 106 N-terminal amino acids of von Willebrand Factor of the epitope corresponding to a monoclonal antibody which inhibits vWF binding to Factor VIII. Biochem Biophys Res Commun 1989; 163: 618-26
- 42 Bahou WF, Ginsburg D, Sikkink R, Litwiller R, Fass DN. A monoclonal antibody to von Willebrand Factor (vWF) inhibits Factor VIII binding. Localization of its antigenic determinant to a nonadecapeptide at the amino terminus of the mature vWF polypeptide. J Clin Invest 1989; 84: 56-61
- 43 Fay PJ, Coumans JV, Walker FJ. von Willebrand Factor mediates protection of Factor VIII from activated protein C-catalyzed inactivation. J Biol Chem 1991; 266: 2172-7
- 44 Sobel M, Mcneill PM, Carlson PL, Kermode JC, Adelman B, Conroy R, Marques D. Heparin inhibition of von Willebrand factor-dependent platelet function in vitro and in vivo . J Clin Invest 1991; 87: 1787-93
- 45 Fujimura Y, Titani K, Holland LZ, Russel SR, Roberts JR, Elder JH, Ruggeri ZM, Zimmerman TS. 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 1986; 261: 381-5
- 46 Weinstein M, Vosburgh E, Phillips M, Turner N, Chute-Rose L, Moake J. Isolation from commercial aurintricarboxylic acid of the most effective polymeric inhibitors of von Willebrand factor. Interaction with platelet glycoprotein Ib. Comparison with other polyanionic and polyaromatic polymers. Blood 1991; 78: 2291-8
- 47 Fujimura Y, Titani K, Holland LZ, Roberts JR, Kostel P, Ruggeri ZM, Zimmerman TS. A heparin-binding domain of human von Willebrand factor. Characterization and localization of a tryptic fragment extending from amino acid residue Val449 to Lys728 . J Biol Chem 1987; 262: 1734-9
- 48 Mohri H, Yoshioka A, Zimmerman TS, Ruggeri ZM. 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 1989; 264: 17361-7
- 49 Sugimoto M, Mohri H, McClintock RA, Ruggeri ZM. 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 1991; 266: 18172-8
- 50 Sixma JJ, Schiphorst ME, Verweij CL, Pannekoek H. Effect of deletion of the A1-domain of von Willebrand factor on its binding to heparin, collagen and platelets in the presence of ristocetin. Eur J Biochem 1991; 196: 369-75
- 51 Pietu G, Meulien P, Cherel G, Courtney M, Meyer D. Production in Escherichia Coli of a biologically active subfragment of von Willebrand Factor corresponding to the platelet glycoprotein Ib collagen and heparin binding domains. Biochem Biophys Res Commun 1989; 164: 1339-47
- 52 Sugimoto M, Ricca G, Hrinda ME, Schreiber AB, Searfoss GH, Bottini E, Ruggeri ZM. Functional modulation of the isolated glycoprotein Ib binding domain of von Willebrand factor expressed in Escherichia-Coli . Biochemistry 1991; 30: 5202-9
- 53 Carew JA, Quinn SM, Stoddart JH, Lynch DC. O-linked carbohydrate of recombinant von Willebrand Factor influences ristocetin-induced binding to platelet glycoprotein Ib. J Clin Invest 1992; 90: 2258-67
- 54 Pareti FI, Niiya K, McPherson JM, Ruggeri ZM. Isolation and characterization of two domains of human von Willebrand factor that interact with fibrillar collagen types I and III. J Biol Chem 1987; 262: 13835-41
- 55 Kalafatis M, Takahashi Y, Girma JP, Meyer D. Localization of a collagen-interactive domain of human von Willebrand Factor between amino acid residues GIy911 and Glu1365 . Blood 1987; 70: 1577-83
- 56 Roth GJ, Titani K, Hoyer LW, Hickey MJ. Localization of binding sites within human von Willebrand factor for monomeric type III collagen. Biochemistry 1986; 25: 8357-61
- 57 Fretto LJ, Fowler WE, McCaslin DR, Enckson HP, McKee PA. Substructure of human von Willebrand Factor. Proteolysis by V-8 and characterization of two functional domains. J Biol Chem 1986; 261: 15679-89
- 58 Sobel M, Soler DF, Kermode JC, Harris RB. Localization and characterization of a heparin binding domain peptide of human von Willebrand factor. J Biol Chem 1992; 267: 8857-62
- 59 Christophe O, Obert B, Meyer D, Girma JP. The binding domain of yon Willebrand Factor to sulfatides is distinct from those interacting with glycoprotein Ib heparin, and collagen and resides between amino acid residues Leu512 and Lys673 . Blood 1991; 78: 2310-7
- 60 Berndt MC, Booth WJ, Andrews RK, Castaldi PA. Definition of von Willebrand Factor (vWF)-GPIb-IX complex interaction using vWF-based peptides. Thromb Haemoslas 1991; 65: 748 (abstr)
- 61 Mohri H, Fujimura Y, Shima M, Yoshioka A, Houghten RA, Ruggeri ZM, Zimmerman TS. Structure of the von Willebrand Factor domain interacting with glycoprotein Ib. J Biol Chem 1988; 263: 17901-4
- 62 Berndt MC, Ward CM, Booth WJ, Castaldi PA, Mazurov AV, Andrews RK. Identification of aspartic acid 514 through glutamic acid 542 as a glycoprotein Ib-IX complex receptor recognition sequence in von Willebrand factor—Mechanism of modulation of von Willebrand factor by ristocetin and botrocetin. Biochemistry 1992; 31: 11144-51
- 63 Girma JP, Takahashi Y, Yoshioka A, Diaz J, Meyer D. Ristocetin and botrocetin involve two distinct domains of von Willebrand factor for binding to platelet membrane glycoprotein Ib. Thromb Haemostas 1990; 64: 326-32
- 64 Rabinowitz I, Tuley EA, Mancuso DJ, Randi AM, Firkin BG, Howard MA, Sadler JE. von Willebrand disease type-B—A missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib. Proc Natl Acad Sci USA 1992; 89: 9846-9
- 65 Azuma H, Sugimoto M, Ruggeri ZM, Ware J. A role of von Willebrand Factor proline residues 702-704 in ristocetin-mediated binding to platelet glycoprotein lb. Thromb Haemostas 1993; 69: 192-6
- 66 Girma JP, Fressinaud E, Christophe O, Rouault C, Obert B, Takahashi Y, Meyer D. Aurin tricarboxylic acid inhibits platelet adhesion to collagen by binding to the 509-695 disulphide loop of von Willebrand factor competing with glycoprotein Ib. Thromb Haemostas 1992; 68: 707-13
- 67 Bahnak BR, Lavergne JM, Ferreira V, Kerbiriou Nabias D, Meyer D. Comparison of the primary structure of the functional domains of human and porcine von Willebrand factor that mediate platelet adhesion. Biochem Biophys Res Commun 1992; 182: 561-8
- 68 Bakhshi MR, Myers JC, Howard PS, Soprano DR, Kirby EP. Sequencing of the primary adhesion domain of bovine von Willebrand factor. Biochim Biophys Acta 1992; 1132: 325-8
- 69 Lavergne JM, de Paillette L, Bahnak BR, Ribba AS, Fressinaud E, Meyer D, Pietu G. Defects in type IIA von Willebrand disease - A cysteine 509 to arginine substitution in the mature von Willebrand factor disrupts a disulphide loop involved in the interaction with platelet glycoprotein Ib-IX. Br J Haematol 1992; 82: 66-72
- 70 Lyons SE, Bruck ME, Bowie EJW, Ginsburg D. Impaired intracellular transport produced by a subset of type IIA von Willebrand disease mutations. J Biol Chem 1992; 267: 4424-30
- 71 Ribba AS, Voorberg J, Meyer D, Pannekoek H, Pietu G. Characterization of recombinant von Willebrand factor corresponding to mutations in type IIA and type IIB von Willebrand disease. J Biol Chem 1992; 267: 23209-15
- 72 Girma JP, Kalafatis M, Pietu G, Lavergne JM, Chopek MW, Edgington TS, Meyer D. Mapping of distinct von Willebrand Factor domains interacting with platelet GPIb and GPIIb/IIIa and with collagen using monoclonal antibodies. Blood 1986; 67: 1356-66
- 73 Plow EF, Pierschbachcr MD, Ruoslahti L, Margueric GA, Ginsberg MH. The effect of Arg-Gly-Asp-containing peptides on fibrinogen and von Willebrand Factor binding to platelets. Proc Natl Acad Sci USA 1985; 82: 8057-61
- 74 Beacham DA, Wise RJ, Turci SM, Handin RI. Selective inactivation of the Arg-Gly-Asp-Ser (RGDS) binding site in von Willebrand Factor by site-directed mutagenesis. J Biol Chem 1992; 79: 2039-47
- 75 Berliner S, Niiya K, Roberts JR, Houghten RA, Ruggeri ZM. 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 Arg1774 Gly1745-Asp1746 . J Biol Chem 1988; 263: 7500-5
- 76 Fressinaud E, Girma JP, Sadler JE, Baumgartncr HR, Meyer D. Synthetic RGDS-containing peptides of von Willebrand Factor inhibit platelet adhesion to collagen. Thromb Haemoslas 1990; 64: 589-93
- 77 Trapani Lombardo V, Hodson E, Roberts JR, Kunicki TJ, Zimmerman TS, Ruggeri ZM. 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 1985; 76: 1950-8