• 1
    Sadler JE, Budde U, Eikenboom JC, Favaloro EJ, Hill FG, Holmberg L, Ingerslev J, Lee CA, Lillicrap D, Mannucci PM, Mazurier C, Meyer D, Nichols WL, Nishino M, Peake IR, Rodeghiero F, Schneppenheim R, Ruggeri ZM, Srivastava A, Montgomery RR, 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: 210314.
  • 2
    James PD, Lillicrap D. von Willebrand disease: clinical and laboratory lessons learned from the large von Willebrand disease studies. Am J Hematol 2012; 87(Suppl. 1): S411.
  • 3
    James PD, Goodeve AC. von Willebrand disease. Genet Med 2011; 13: 36576.
  • 4
    Schneppenheim R, Budde U. von Willebrand factor: the complex molecular genetics of a multidomain and multifunctional protein. J Thromb Haemost 2011; 9(Suppl. 1): 20915.
  • 5
    Sadler JE. Low von Willebrand factor: sometimes a risk factor and sometimes a disease. Hematology Am Soc Hematol Educ Program 2009; 10612.
  • 6
    Rodeghiero F, Castaman G, Tosetto A. How I treat von Willebrand disease. Blood 2009; 114: 115865.
  • 7
    Abshire TC, Federici AB, Alvarez MT, Bowen J, Carcao MD, Cox Gill J, Key NS, Kouides PA, Kurnik K, Lail AE, Leebeek FW, Makris M, Mannucci PM, Winikoff R, Berntorp E. Prophylaxis in severe forms of von Willebrand’s disease: results from the von Willebrand Disease Prophylaxis Network (VWD PN). Haemophilia 2012; doi: 10.1111/j.1365-2516.2012.02916.x.
  • 8
    De Meyer SF, Deckmyn H, Vanhoorelbeke K. von Willebrand factor to the rescue. Blood 2009; 113: 504957.
  • 9
    Westrick RJ, Ginsburg D. Modifier genes for disorders of thrombosis and hemostasis. J Thromb Haemost 2009; 7(Suppl. 1): 1325.
  • 10
    Lenting PJ, de Groot PG, De Meyer SF, Vanhoorelbeke K, Pruss C, Lillicrap D, Marx I, Denis CV. Correction of the bleeding time in von Willebrand factor (VWF)-deficient mice using murine VWF. Blood 2007; 109: 22678.
  • 11
    Rayes J, Hollestelle MJ, Legendre P, Marx I, de Groot PG, Christophe OD, Lenting PJ, Denis CV. Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B. Blood 2010; 115: 48707.
  • 12
    Golder M, Pruss CM, Hegadorn C, Mewburn J, Laverty K, Sponagle K, Lillicrap D. Mutation-specific hemostatic variability in mice expressing common type 2B von Willebrand disease substitutions. Blood 2010; 115: 48629.
  • 13
    Pruss CM, Golder M, Bryant A, Hegadorn CA, Burnett E, Laverty K, Sponagle K, Dhala A, Notley C, Haberichter S, Lillicrap D. Pathologic mechanisms of type 1 VWD mutations R1205H and Y1584C through in vitro and in vivo mouse models. Blood 2011; 117: 435866.
  • 14
    Pruss CM, Golder M, Bryant A, Hegadorn C, Haberichter S, Lillicrap D. Use of a mouse model to elucidate the phenotypic effects of the von Willebrand factor cleavage mutants, Y1605A/M1606A and R1597W. J Thromb Haemost 2012; 10: 94050.
  • 15
    Pannekoek H, Voorberg J. Molecular cloning, expression and assembly of multimeric von Willebrand factor. Baillieres Clin Haematol 1989; 2: 87996.
  • 16
    Springer TA. Biology and physics of von Willebrand factor concatamers. J Thromb Haemost 2011; 9(Suppl. 1): 13043.
  • 17
    Zhou YF, Eng ET, Nishida N, Lu C, Walz T, Springer TA. A pH-regulated dimeric bouquet in the structure of von Willebrand factor. EMBO J 2011; 30: 4098111.
  • 18
    Zhou YF, Eng ET, Zhu J, Lu C, Walz T, Springer TA. Sequence and structure relationships within von Willebrand factor. Blood 2012; 120: 44958.
  • 19
    Siedlecki CA, Lestini BJ, Kottke-Marchant KK, Eppell SJ, Wilson DL, Marchant RE. Shear-dependent changes in the three-dimensional structure of human von Willebrand factor. Blood 1996; 88: 293950.
  • 20
    Schneider SW, Nuschele S, Wixforth A, Gorzelanny C, Alexander-Katz A, Netz RR, Schneider MF. Shear-induced unfolding triggers adhesion of von Willebrand factor fibers. Proc Natl Acad Sci USA 2007; 104: 7899903.
  • 21
    Zhang X, Halvorsen K, Zhang CZ, Wong WP, Springer TA. Mechanoenzymatic cleavage of the ultralarge vascular protein von Willebrand factor. Science 2009; 324: 13304.
  • 22
    Ruggeri ZM, Orje JN, Habermann R, Federici AB, Reininger AJ. Activation-independent platelet adhesion and aggregation under elevated shear stress. Blood 2006; 108: 190310.
  • 23
    Ganderton T, Berndt MC, Chesterman CN, Hogg PJ. Hypothesis for control of von Willebrand factor multimer size by intra-molecular thiol-disulphide exchange. J Thromb Haemost 2007; 5: 2046.
  • 24
    Li Y, Choi H, Zhou Z, Nolasco L, Pownall HJ, Voorberg J, Moake JL, Dong JF. Covalent regulation of ULVWF string formation and elongation on endothelial cells under flow conditions. J Thromb Haemost 2008; 6: 113543.
  • 25
    Yeh HC, Zhou Z, Choi H, Tekeoglu S, May W 3rd, Wang C, Turner N, Scheiflinger F, Moake JL, Dong JF. Disulfide bond reduction of von Willebrand factor by ADAMTS-13. J Thromb Haemost 2010; 8: 277888.
  • 26
    Fu X, Chen J, Gallagher R, Zheng Y, Chung DW, Lopez JA. Shear stress-induced unfolding of von Willebrand factor accelerates oxidation of key methionine residues in the A1A2A3 region. Blood 2011; 118: 528391.
  • 27
    Chen J, Fu X, Wang Y, Ling M, McMullen B, Kulman J, Chung DW, Lopez JA. Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13. Blood 2010; 115: 70612.
  • 28
    Lancellotti S, De Filippis V, Pozzi N, Peyvandi F, Palla R, Rocca B, Rutella S, Pitocco D, Mannucci PM, De Cristofaro R. Formation of methionine sulfoxide by peroxynitrite at position 1606 of von Willebrand factor inhibits its cleavage by ADAMTS-13: a new prothrombotic mechanism in diseases associated with oxidative stress. Free Radic Biol Med 2010; 48: 44656.
  • 29
    Rastegarlari G, Pegon JN, Casari C, Odouard S, Navarrete AM, Saint-Lu N, van Vlijmen BJ, Legendre P, Christophe OD, Denis CV, Lenting PJ. Macrophage LRP1 contributes to the clearance of von Willebrand factor. Blood 2012; 119: 212634.
  • 30
    Castro-Nunez L, Dienava-Verdoold I, Herczenik E, Mertens K, Meijer AB. Shear stress is required for the endocytic uptake of the factor VIII–von Willebrand factor complex by macrophages. J Thromb Haemost 2012; 10: 192937.
  • 31
    Koster T, Blann AD, Briet E, Vandenbroucke JP, Rosendaal FR. Role of clotting factor VIII in effect of von Willebrand factor on occurrence of deep-vein thrombosis. Lancet 1995; 345: 1525.
  • 32
    Lenting PJ, van Mourik JA, Mertens K. The life cycle of coagulation factor VIII in view of its structure and function. Blood 1998; 92: 398396.
  • 33
    Campos M, Buchanan A, Yu F, Barbalic M, Xiao Y, Chambless LE, Wu KK, Folsom AR, Boerwinkle E, Dong JF. Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study. Blood 2012; 119: 192934.
  • 34
    Lijfering WM, Rosendaal FR, Cannegieter SC. Risk factors for venous thrombosis – current understanding from an epidemiological point of view. Br J Haematol 2010; 149: 82433.
  • 35
    Smith NL, Rice KM, Bovill EG, Cushman M, Bis JC, McKnight B, Lumley T, Glazer NL, van Hylckama Vlieg A, Tang W, Dehghan A, Strachan DP, O’Donnell CJ, Rotter JI, Heckbert SR, Psaty BM, Rosendaal FR. Genetic variation associated with plasma von Willebrand factor levels and the risk of incident venous thrombosis. Blood 2011; 117: 600711.
  • 36
    Wetzstein V, Budde U, Oyen F, Ding X, Herrmann J, Liebig B, Schneppenheim R. Intracranial hemorrhage in a term newborn with severe von Willebrand disease type 3 associated with sinus venous thrombosis. Haematologica 2006; 91(Suppl. 12): ECR60.
  • 37
    Tsai AW, Cushman M, Rosamond WD, Heckbert SR, Tracy RP, Aleksic N, Folsom AR. Coagulation factors, inflammation markers, and venous thromboembolism: the longitudinal investigation of thromboembolism etiology (LITE). Am J Med 2002; 113: 63642.
  • 38
    Takahashi M, Yamashita A, Moriguchi-Goto S, Marutsuka K, Sato Y, Yamamoto H, Koshimoto C, Asada Y. Critical role of von Willebrand factor and platelet interaction in venous thromboembolism. Histol Histopathol 2009; 24: 13918.
  • 39
    Yamamoto H, Vreys I, Stassen JM, Yoshimoto R, Vermylen J, Hoylaerts MF. Antagonism of vWF inhibits both injury induced arterial and venous thrombosis in the hamster. Thromb Haemost 1998; 79: 20210.
  • 40
    Chauhan AK, Kisucka J, Lamb CB, Bergmeier W, Wagner DD. von Willebrand factor and factor VIII are independently required to form stable occlusive thrombi in injured veins. Blood 2007; 109: 24249.
  • 41
    Brill A, Fuchs TA, Chauhan AK, Yang JJ, De Meyer SF, Kollnberger M, Wakefield TW, Lammle B, Massberg S, Wagner DD. von Willebrand factor-mediated platelet adhesion is critical for deep vein thrombosis in mouse models. Blood 2011; 117: 14007.
  • 42
    Fuchs TA, Brill A, Duerschmied D, Schatzberg D, Monestier M, Myers DD Jr, Wrobleski SK, Wakefield TW, Hartwig JH, Wagner DD. Extracellular DNA traps promote thrombosis. Proc Natl Acad Sci USA 2010; 107: 158805.
  • 43
    Fuchs TA, Brill A, Wagner DD. Neutrophil extracellular trap (NET) impact on deep vein thrombosis. Arterioscler Thromb Vasc Biol 2012; 32: 177783.
  • 44
    Brill A, Fuchs TA, Savchenko AS, Thomas GM, Martinod K, De Meyer SF, Bhandari AA, Wagner DD. Neutrophil extracellular traps promote deep vein thrombosis in mice. J Thromb Haemost 2012; 10: 13644.
  • 45
    De Meyer SF, Stoll G, Wagner DD, Kleinschnitz C. von Willebrand factor: an emerging target in stroke therapy. Stroke 2012; 43: 599606.
  • 46
    van Schie MC, van Loon JE, de Maat MP, Leebeek FW. Genetic determinants of von Willebrand factor levels and activity in relation to the risk of cardiovascular disease: a review. J Thromb Haemost 2011; 9: 899908.
  • 47
    Kleinschnitz C, De Meyer SF, Schwarz T, Austinat M, Vanhoorelbeke K, Nieswandt B, Deckmyn H, Stoll G. Deficiency of von Willebrand factor protects mice from ischemic stroke. Blood 2009; 113: 36003.
  • 48
    Zhao BQ, Chauhan AK, Canault M, Patten IS, Yang JJ, Dockal M, Scheiflinger F, Wagner DD. von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke. Blood 2009; 114: 332934.
  • 49
    Wagner DD. The Weibel–Palade body: the storage granule for von Willebrand factor and P-selectin. Thromb Haemost 1993; 70: 10510.
  • 50
    Denis CV, Andre P, Saffaripour S, Wagner DD. Defect in regulated secretion of P-selectin affects leukocyte recruitment in von Willebrand factor-deficient mice. Proc Natl Acad Sci USA 2001; 98: 40727.
  • 51
    Pendu R, Terraube V, Christophe OD, Gahmberg CG, de Groot PG, Lenting PJ, Denis CV. P-selectin glycoprotein ligand 1 and beta2-integrins cooperate in the adhesion of leukocytes to von Willebrand factor. Blood 2006; 108: 374652.
  • 52
    Pegon JN, Kurdi M, Casari C, Odouard S, Denis CV, Christophe OD, Lenting PJ. Factor VIII and von Willebrand factor are ligands for the carbohydrate-receptor Siglec-5. Haematologica 2012. (in press).
  • 53
    Bernardo A, Ball C, Nolasco L, Choi H, Moake JL, Dong JF. Platelets adhered to endothelial cell-bound ultra-large von Willebrand factor strings support leukocyte tethering and rolling under high shear stress. J Thromb Haemost 2005; 3: 56270.
  • 54
    Petri B, Broermann A, Li H, Khandoga AG, Zarbock A, Krombach F, Goerge T, Schneider SW, Jones C, Nieswandt B, Wild MK, Vestweber D. von Willebrand factor promotes leukocyte extravasation. Blood 2010; 116: 471219.
  • 55
    Methia N, Andre P, Denis CV, Economopoulos M, Wagner DD. Localized reduction of atherosclerosis in von Willebrand factor-deficient mice. Blood 2001; 98: 14248.
  • 56
    Noubade R, del Rio R, McElvany B, Zachary JF, Millward JM, Wagner DD, Offner H, Blankenhorn EP, Teuscher C. von-Willebrand factor influences blood brain barrier permeability and brain inflammation in experimental allergic encephalomyelitis. Am J Pathol 2008; 173: 892900.
  • 57
    Nichols TC, Bellinger DA, Tate DA, Reddick RL, Read MS, Koch GG, Brinkhous KM, Griggs TR. von Willebrand factor and occlusive arterial thrombosis. A study in normal and von Willebrand’s disease pigs with diet-induced hypercholesterolemia and atherosclerosis. Arteriosclerosis 1990; 10: 44961.
  • 58
    van Galen KP, Tuinenburg A, Smeets EM, Schutgens RE. von Willebrand factor deficiency and atherosclerosis. Blood Rev 2012; 26: 18996.
  • 59
    Dole VS, Bergmeier W, Mitchell HA, Eichenberger SC, Wagner DD. Activated platelets induce Weibel–Palade-body secretion and leukocyte rolling in vivo: role of P-selectin. Blood 2005; 106: 23349.
  • 60
    Chauhan AK, Kisucka J, Brill A, Walsh MT, Scheiflinger F, Wagner DD. ADAMTS13: a new link between thrombosis and inflammation. J Exp Med 2008; 205: 206574.
  • 61
    Gandhi C, Khan MM, Lentz SR, Chauhan AK. ADAMTS13 reduces vascular inflammation and the development of early atherosclerosis in mice. Blood 2012; 119: 238591.
  • 62
    Fujioka M, Hayakawa K, Mishima K, Kunizawa A, Irie K, Higuchi S, Nakano T, Muroi C, Fukushima H, Sugimoto M, Banno F, Kokame K, Miyata T, Fujiwara M, Okuchi K, Nishio K. ADAMTS13 gene deletion aggravates ischemic brain damage: a possible neuroprotective role of ADAMTS13 by ameliorating postischemic hypoperfusion. Blood 2010; 115: 16503.
  • 63
    Khan MM, Motto DG, Lentz SR, Chauhan AK. ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice. J Thromb Haemost 2012; 10: 166571.
  • 64
    Buzza MS, Dyson JM, Choi H, Gardiner EE, Andrews RK, Kaiserman D, Mitchell CA, Berndt MC, Dong JF, Bird PI. Antihemostatic activity of human granzyme B mediated by cleavage of von Willebrand factor. J Biol Chem 2008; 283: 22498504.
  • 65
    Raife TJ, Cao W, Atkinson BS, Bedell B, Montgomery RR, Lentz SR, Johnson GF, Zheng XL. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood 2009; 114: 166674.
  • 66
    Hollestelle MJ, Lai KW, van Deuren M, Lenting PJ, de Groot PG, Sprong T, Bovenschen N. Cleavage of von Willebrand factor by granzyme M destroys its factor VIII binding capacity. PLoS One 2011; 6: e24216.
  • 67
    Haberichter SL, Fahs SA, Montgomery RR. von Willebrand factor storage and multimerization: 2 independent intracellular processes. Blood 2000; 96: 180815.
  • 68
    Leyte A, Voorberg J, Van Schijndel HB, Duim B, Pannekoek H, Van Mourik JA. The pro-polypeptide of von Willebrand factor is required for the formation of a functional factor VIII-binding site on mature von Willebrand factor. Biochem J 1991; 274: 25761.
  • 69
    Hannah MJ, Skehel P, Erent M, Knipe L, Ogden D, Carter T. Differential kinetics of cell surface loss of von Willebrand factor and its propolypeptide after secretion from Weibel–Palade bodies in living human endothelial cells. J Biol Chem 2005; 280: 2282730.
  • 70
    Madabhushi SR, Shang C, Dayananda KM, Rittenhouse-Olson K, Murphy M, Ryan TE, Montgomery RR, Neelamegham S. von Willebrand factor (VWF) propeptide binding to VWF D′D3 domain attenuates platelet activation and adhesion. Blood 2012; 119: 476978.
  • 71
    Bendetowicz AV, Morris JA, Wise RJ, Gilbert GE, Kaufman RJ. Binding of factor VIII to von Willebrand factor is enabled by cleavage of the von Willebrand factor propeptide and enhanced by formation of disulfide-linked multimers. Blood 1998; 92: 52938.
  • 72
    Crawley JT, de Groot R, Xiang Y, Luken BM, Lane DA. Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor. Blood 2011; 118: 321221.
  • 73
    Shahbazi S, Lenting PJ, Fribourg C, Terraube V, Denis CV, Christophe OD. Characterization of the interaction between von Willebrand factor and osteoprotegerin. J Thromb Haemost 2007; 5: 195662.
  • 74
    Saint-Lu N, Oortwijn BD, Pegon JN, Odouard S, Christophe OD, de Groot PG, Denis CV, Lenting PJ. Identification of galectin-1 and galectin-3 as novel partners for von Willebrand factor. Arterioscler Thromb Vasc Biol 2012; 32: 894901.
  • 75
    Mannucci PM, Capoferri C, Canciani MT. Plasma levels of von Willebrand factor regulate ADAMTS-13, its major cleaving protease. Br J Haematol 2004; 126: 21318.
  • 76
    Furlan M, Robles R, Morselli B, Sandoz P, Lammle B. Recovery and half-life of von Willebrand factor-cleaving protease after plasma therapy in patients with thrombotic thrombocytopenic purpura. Thromb Haemost 1999; 81: 813.
  • 77
    Holmberg HL, Kjalke M, Karpf D, Hilden I, Pelzer H, Koefoed-Hansen M, Johnsen L, Thim L, Karlsson J, Jespersgaard C, Bolt G, Stennicke H. High affinity binding of FVIII to VWF is not required for the haemostatic effect of FVIII in vivo. ASH Annual Meeting Abstracts 2011; 118: 1182.
  • 78
    Cao W, Krishnaswamy S, Camire RM, Lenting PJ, Zheng XL. Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc Natl Acad Sci USA 2008; 105: 741621.
  • 79
    Liu FT, Yang RY, Hsu DK. Galectins in acute and chronic inflammation. Ann NY Acad Sci 2012; 1253: 8091.
  • 80
    Le Mercier M, Fortin S, Mathieu V, Kiss R, Lefranc F. Galectins and gliomas. Brain Pathol 2010; 20: 1727.
  • 81
    Canobbio I, Balduini C, Torti M. Signalling through the platelet glycoprotein Ib–V–IX complex. Cell Signal 2004; 16: 132944.
  • 82
    Starke RD, Ferraro F, Paschalaki KE, Dryden NH, McKinnon TA, Sutton RE, Payne EM, Haskard DO, Hughes AD, Cutler DF, Laffan MA, Randi AM. Endothelial von Willebrand factor regulates angiogenesis. Blood 2011; 117: 107180.
  • 83
    Pi L, Shenoy AK, Liu J, Kim S, Nelson N, Xia H, Hauswirth WW, Petersen BE, Schultz GS, Scott EW. CCN2/CTGF regulates neovessel formation via targeting structurally conserved cystine knot motifs in multiple angiogenic regulators. FASEB J 2012; 26: 336579.
  • 84
    van Breevoort D, van Agtmaal EL, Dragt BS, Gebbinck JK, Dienava-Verdoold I, Kragt A, Bierings R, Horrevoets AJ, Valentijn KM, Eikenboom JC, Fernandez-Borja M, Meijer AB, Voorberg J. Proteomic screen identifies IGFBP7 as a novel component of endothelial cell-specific Weibel–Palade bodies. J Proteome Res 2012; 11: 292536.
  • 85
    Park SO, Wankhede M, Lee YJ, Choi EJ, Fliess N, Choe SW, Oh SH, Walter G, Raizada MK, Sorg BS, Oh SP. Real-time imaging of de novo arteriovenous malformation in a mouse model of hereditary hemorrhagic telangiectasia. J Clin Invest 2009; 119: 348796.
  • 86
    Fressinaud E, Meyer D. International survey of patients with von Willebrand disease and angiodysplasia. Thromb Haemost 1993; 70: 546.
  • 87
    Castaman G, Federici AB, Tosetto A, La Marca S, Stufano F, Mannucci PM, Rodeghiero F. Different bleeding risk in type 2A and 2M Von Willebrand disease: a two-year prospective study in 107 patients. J Thromb Haemost 2012; 10: 6328.
  • 88
    Vincentelli A, Susen S, Le Tourneau T, Six I, Fabre O, Juthier F, Bauters A, Decoene C, Goudemand J, Prat A, Jude B. Acquired von Willebrand syndrome in aortic stenosis. N Engl J Med 2003; 349: 3439.
  • 89
    Slaughter MS. Hematologic effects of continuous flow left ventricular assist devices. J Cardiovasc Transl Res 2010; 3: 61824.
  • 90
    Bosmans JM, Kockx MM, Vrints CJ, Bult H, De Meyer GR, Herman AG. Fibrin(ogen) and von Willebrand factor deposition are associated with intimal thickening after balloon angioplasty of the rabbit carotid artery. Arterioscler Thromb Vasc Biol 1997; 17: 63445.
  • 91
    Qin F, Impeduglia T, Schaffer P, Dardik H. Overexpression of von Willebrand factor is an independent risk factor for pathogenesis of intimal hyperplasia: preliminary studies. J Vasc Surg 2003; 37: 4339.
  • 92
    Zhang X, Meng H, Blaivas M, Rushing EJ, Moore BE, Schwartz J, Lopes MB, Worrall BB, Wang MM. von Willebrand factor permeates small vessels in CADASIL and inhibits smooth muscle gene expression. Transl Stroke Res 2012; 3: 13845.
  • 93
    Li S, Wang Z, Liao Y, Zhang W, Shi Q, Yan R, Ruan C, Dai K. The glycoprotein Ibalpha–von Willebrand factor interaction induces platelet apoptosis. J Thromb Haemost 2010; 8: 34150.
  • 94
    Terraube V, Pendu R, Baruch D, Gebbink MF, Meyer D, Lenting PJ, Denis CV. Increased metastatic potential of tumor cells in von Willebrand factor-deficient mice. J Thromb Haemost 2006; 4: 51926.
  • 95
    Terraube V, Marx I, Denis CV. Role of von Willebrand factor in tumor metastasis. Thromb Res 2007; 120(Suppl. 2): S6470.
  • 96
    Mochizuki S, Soejima K, Shimoda M, Abe H, Sasaki A, Okano HJ, Okano H, Okada Y. Effect of ADAM28 on carcinoma cell metastasis by cleavage of von Willebrand factor. J Natl Cancer Inst 2012; 104: 90622.
  • 97
    Wise RJ, Pittman DD, Handin RI, Kaufman RJ, Orkin SH. The propeptide of von Willebrand factor independently mediates the assembly of von Willebrand multimers. Cell 1988; 52: 22936.
  • 98
    Michaux G, Pullen TJ, Haberichter SL, Cutler DF. P-selectin binds to the D′-D3 domains of von Willebrand factor in Weibel–Palade bodies. Blood 2006; 107: 39224.
  • 99
    Fujimura Y, Titani K, Holland LZ, Russell 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: 3815.
  • 100
    Hulstein JJ, Lenting PJ, de Laat B, Derksen RH, Fijnheer R, de Groot PG. beta2-Glycoprotein I inhibits von Willebrand factor dependent platelet adhesion and aggregation. Blood 2007; 110: 148391.
  • 101
    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: 398406.
  • 102
    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: 1383541.
  • 103
    Pimanda JE, Ganderton T, Maekawa A, Yap CL, Lawler J, Kershaw G, Chesterman CN, Hogg PJ. Role of thrombospondin-1 in control of von Willebrand factor multimer size in mice. J Biol Chem 2004; 279: 2143948.
  • 104
    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; 267: 340915.
  • 105
    Keuren JF, Baruch D, Legendre P, Denis CV, Lenting PJ, Girma JP, Lindhout T. von Willebrand factor C1C2 domain is involved in platelet adhesion to polymerized fibrin at high shear rate. Blood 2004; 103: 17416.
  • 106
    Bierings R, van den Biggelaar M, Kragt A, Mertens K, Voorberg J, van Mourik JA. Efficiency of von Willebrand factor-mediated targeting of interleukin-8 into Weibel–Palade bodies. J Thromb Haemost 2007; 5: 251219.