Targeting von Willebrand factor and platelet glycoprotein Ib receptor

References

• Lowenberg EC, Meijers JC, Levi M. Platelet–vessel wall interaction in health and disease. Neth. J. Med.68(6), 242–251 (2010).
   PubMed Web of Science ®Google Scholar
• Blann A. Von Willebrand factor and the endothelium in vascular disease. Br. J. Biomed. Sci.50(2), 125–134 (1993).
   PubMed Web of Science ®Google Scholar
• Ruggeri ZM, Ware J. Von Willebrand factor. FASEB J.7(2), 308–316 (1993).
   PubMed Web of Science ®Google Scholar
• Spiel AO, Gilbert JC, Jilma B. Von Willebrand factor in cardiovascular disease: focus on acute coronary syndromes. Circulation117(11), 1449–1459 (2008).
   PubMed Web of Science ®Google Scholar
• Sadler JE. Biochemistry and genetics of von Willebrand factor. Annu. Rev. Biochem.67, 395–424 (1998).
   PubMed Web of Science ®Google Scholar
• Hoylaerts MF. Platelet–vessel wall interactions in thrombosis and restenosis role of von Willebrand factor. Verh. K Acad. Geneeskd. Belg.59(3), 161–183 (1997).
   PubMedGoogle Scholar
• Pareti FI, Fujimura Y, Dent JA, Holland LZ, Zimmerman TS, Ruggeri ZM. Isolation and characterization of a collagen binding domain in human von Willebrand factor. J. Biol. Chem.261(32), 15310–15315 (1986).
   PubMed Web of Science ®Google Scholar
• Siedlecki CA, Lestini BJ, Kottke-Marchant KK et al. Shear-dependent changes in the three-dimensional structure of human von Willebrand factor. Blood88(8), 2939–2950 (1996).
   PubMed Web of Science ®Google Scholar
• Matsushita T, Sadler JE. Identification of amino acid residues essential for von Willebrand factor binding to platelet glycoprotein Ib. Charged-to-alanine scanning mutagenesis of the A1 domain of human von Willebrand factor. J. Biol. Chem.270(22), 13406–13414 (1995).
   PubMed Web of Science ®Google Scholar
• Reininger AJ, Heijnen HF, Schumann H, Specht HM, Schramm W, Ruggeri ZM. Mechanism of platelet adhesion to von Willebrand factor and microparticle formation under high shear stress. Blood107(9), 3537–3545 (2006).
   PubMed Web of Science ®Google Scholar
• Nichols WL, Hultin MB, James AH et al. von Willebrand disease (vWD): evidence-based diagnosis and management guidelines, the National Heart, Lung, and Blood Institute (NHLBI) Expert Panel report (USA). Haemophilia14(2), 171–232 (2008).
   PubMed Web of Science ®Google Scholar
• Savage B, Saldivar E, Ruggeri ZM. Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor. Cell84(2), 289–297 (1996).
   PubMed Web of Science ®Google Scholar
• Fredrickson BJ, Dong JF, McIntire LV, Lopez JA. Shear-dependent rolling on von Willebrand factor of mammalian cells expressing the platelet glycoprotein Ib–IX–V complex. Blood92(10), 3684–3693 (1998).
   PubMed Web of Science ®Google Scholar
• Pendu R, Christophe OD, Denis CV. Mouse models of von Willebrand disease. J. Thromb. Haemost.7(Suppl. 1) 61–64 (2009).
   PubMed Web of Science ®Google Scholar
• Denis CV, Wagner DD. Platelet adhesion receptors and their ligands in mouse models of thrombosis. Arterioscler. Thromb. Vasc. Biol.27(4), 728–739 (2007).
   PubMed Web of Science ®Google Scholar
• Denis CV, Marx I, Badirou I, Pendu R, Christophe O. Mouse models to study von Willebrand factor structure-function relationships in vivo. Hamostaseologie29(1), 17–18, 20 (2009).
   PubMed Web of Science ®Google Scholar
• Marx I, Christophe OD, Lenting PJ et al. Altered thrombus formation in von Willebrand factor-deficient mice expressing von Willebrand factor variants with defective binding to collagen or GPIIbIIIa. Blood112(3), 603–609 (2008).
   PubMed Web of Science ®Google Scholar
• Vlot AJ, Koppelman SJ, van den Berg MH, Bouma BN, Sixma JJ. The affinity and stoichiometry of binding of human factor VIII to von Willebrand factor. Blood85(11), 3150–3157 (1995).
   PubMed Web of Science ®Google Scholar
• Jaffe EA, Hoyer LW, Nachman RL. Synthesis of von Willebrand factor by cultured human endothelial cells. Proc. Natl Acad. Sci. USA71(5), 1906–1909 (1974).
   PubMed Web of Science ®Google Scholar
• Vischer UM, Wagner DD. Von Willebrand factor proteolytic processing and multimerization precede the formation of Weibel–Palade bodies. Blood83(12), 3536–3544 (1994).
   PubMed Web of Science ®Google Scholar
• Weibel ER, Palade GE. New cytoplasmic components in arterial endothelia. J. Cell Biol.23, 101–112 (1964).
   PubMed Web of Science ®Google Scholar
• Pearson JD. Normal endothelial cell function. Lupus9(3), 183–188 (2000).
   PubMed Web of Science ®Google Scholar
• 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.78(1), 26–30 (1986).
   PubMed Web of Science ®Google Scholar
• Mannucci PM. Platelet von Willebrand factor in inherited and acquired bleeding disorders. Proc. Natl Acad. Sci. USA92(7), 2428–2432 (1995).
   PubMed Web of Science ®Google Scholar
• Nachman R, Levine R, Jaffe EA. Synthesis of factor VIII antigen by cultured guinea pig megakaryocytes. J. Clin. Invest.60(4), 914–921 (1977).
   PubMed Web of Science ®Google Scholar
• Ware RE, Parker RI, McKeown LP, Graham ML. A human chimera for von Willebrand disease following bone marrow transplantation. Am. J. Pediatr. Hematol. Oncol.15(3), 338–342 (1993).
   PubMedGoogle Scholar
• Brouland JP, Egan T, Roussi J et al.In vivo regulation of von willebrand factor synthesis: von Willebrand factor production in endothelial cells after lung transplantation between normal pigs and von Willebrand factor-deficient pigs. Arterioscler. Thromb. Vasc. Biol.19(12), 3055–3062 (1999).
   PubMed Web of Science ®Google Scholar
• Pinsky DJ, Naka Y, Liao H et al. Hypoxia-induced exocytosis of endothelial cell Weibel–Palade bodies. A mechanism for rapid neutrophil recruitment after cardiac preservation. J. Clin. Invest.97(2), 493–500 (1996).
   PubMed Web of Science ®Google Scholar
• Paleolog EM, Crossman DC, McVey JH, Pearson JD. Differential regulation by cytokines of constitutive and stimulated secretion of von Willebrand factor from endothelial cells. Blood75(3), 688–695 (1990).
   PubMed Web of Science ®Google Scholar
• Levine JD, Harlan JM, Harker LA, Joseph ML, Counts RB. Thrombin-mediated release of factor VIII antigen from human umbilical vein endothelial cells in culture. Blood60(2), 531–534 (1982).
   PubMed Web of Science ®Google Scholar
• Chignard M, Balloy V, Renesto P. Leucocyte elastase-mediated release of von Willebrand factor from cultured endothelial cells. Eur. Respir. J.6(6), 791–796 (1993).
   PubMed Web of Science ®Google Scholar
• Jilma B, Pernerstorfer T, Dirnberger E et al. Effects of histamine and nitric oxide synthase inhibition on plasma levels of von Willebrand factor antigen. J. Lab. Clin. Med.131(2), 151–156 (1998).
   PubMed Web of Science ®Google Scholar
• Derhaschnig U, Schweeger-Exeli I, Marsik C et al. Effects of aspirin and NO–aspirin (NCX-4016) on platelet function and coagulation in human endotoxemia. Platelets21(5), 320–328 (2010).
   PubMed Web of Science ®Google Scholar
• Jilma B, Dirnberger E, Eichler HG et al. Partial blockade of nitric oxide synthase blunts the exercise-induced increase of von Willebrand factor antigen and of factor VIII in man. Thromb. Haemost.78(4), 1268–1271 (1997).
   PubMed Web of Science ®Google Scholar
• Rickles FR, Hoyer LW, Rick ME, Ahr DJ. The effects of epinephrine infusion in patients with von Willebrand’s disease. J. Clin. Invest.57(6), 1618–1625 (1976).
   PubMed Web of Science ®Google Scholar
• Mannucci PM, Ruggeri ZM, Pareti FI, Capitanio A. 1-Deamino-8-D-arginine vasopressin: a new pharmacological approach to the management of haemophilia and von Willebrands’ diseases. Lancet1(8017), 869–872 (1977).
   PubMed Web of Science ®Google Scholar
• Richardson M, Tinlin S, De Reske M, Webster S, Senis Y, Giles AR. Morphological alterations in endothelial cells associated with the release of von Willebrand factor after thrombin generation in vivo. Arterioscler. Thromb.14(6), 990–999 (1994).
   PubMed Web of Science ®Google Scholar
• Shim K, Anderson PJ, Tuley EA, Wiswall E, Sadler JE. Platelet–vWF complexes are preferred substrates of ADAMTS13 under fluid shear stress. Blood111(2), 651–657 (2008).
   PubMed Web of Science ®Google Scholar
• Lenting PJ, Pegon JN, Groot E, de Groot PG. Regulation of von Willebrand factor-platelet interactions. Thromb. Haemost.104(3) (2010).
   PubMed Web of Science ®Google Scholar
• Skipwith CG, Cao W, Zheng XL. Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress. J. Biol. Chem.285(37), 28596–28603 (2010).
   PubMed Web of Science ®Google Scholar
• 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. USA105(21), 7416–7421 (2008).
   PubMed Web of Science ®Google Scholar
• 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. Blood100(3), 778–785 (2002).
   PubMed Web of Science ®Google Scholar
• Mulvihill N, Foley JB, Ghaisas N, Murphy R, Crean P, Walsh M. Early temporal expression of soluble cellular adhesion molecules in patients with unstable angina and subendocardial myocardial infarction. Am. J. Cardiol.83(8), 1265–1267, A9 (1999).
   PubMed Web of Science ®Google Scholar
• Thompson SG, Kienast J, Pyke SD, Haverkate F, van de Loo JC. Hemostatic factors and the risk of myocardial infarction or sudden death in patients with angina pectoris. European Concerted Action on Thrombosis and Disabilities Angina Pectoris Study Group. N. Engl. J. Med.332(10), 635–641 (1995).
   PubMed Web of Science ®Google Scholar
• Back LD, Radbill JR, Crawford DW. Analysis of pulsatile, viscous blood flow through diseased coronary arteries of man. J. Biomech.10(5–6), 339–353 (1977).
   PubMed Web of Science ®Google Scholar
• Kroll MH, Hellums JD, McIntire LV, Schafer AI, Moake JL. Platelets and shear stress. Blood88(5), 1525–1541 (1996).
   PubMed Web of Science ®Google Scholar
• Maxwell MJ, Westein E, Nesbitt WS, Giuliano S, Dopheide SM, Jackson SP. Identification of a 2-stage platelet aggregation process mediating shear-dependent thrombus formation. Blood109(2), 566–576 (2007).
   PubMed Web of Science ®Google Scholar
• Mazzucato M, Cozzi MR, Pradella P, Ruggeri ZM, De Marco L. Distinct roles of ADP receptors in von Willebrand factor-mediated platelet signaling and activation under high flow. Blood104(10), 3221–3227 (2004).
   PubMed Web of Science ®Google Scholar
• Gilbert JC, DeFeo-Fraulini T, Hutabarat RM et al. First-in-human evaluation of anti von Willebrand factor therapeutic aptamer ARC1779 in healthy volunteers. Circulation116(23), 2678–2686 (2007).
   PubMed Web of Science ®Google Scholar
• Vanhoorelbeke K, Ulrichts H, Schoolmeester A, Deckmyn H. Inhibition of platelet adhesion to collagen as a new target for antithrombotic drugs. Curr. Drug Targets Cardiovasc. Haematol. Disord.3(2), 125–140 (2003).
   PubMedGoogle Scholar
• Holmberg L, Nilsson IM. Von Willebrand’s disease. Eur. J. Haematol.48(3), 127–141 (1992).
   PubMed Web of Science ®Google Scholar
• 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. Blood109(6), 2424–2429 (2007).
   PubMed Web of Science ®Google Scholar
• Takahashi M, Yamashita A, Moriguchi-Goto S et al. Critical role of von Willebrand factor and platelet interaction in venous thromboembolism. Histol. Histopathol.24(11), 1391–1398 (2009).
   PubMed Web of Science ®Google Scholar
• Montalescot G, Philippe F, Ankri A et al. Early increase of von Willebrand factor predicts adverse outcome in unstable coronary artery disease: beneficial effects of enoxaparin. French Investigators of the ESSENCE Trial. Circulation98(4), 294–299 (1998).
   PubMed Web of Science ®Google Scholar
• Ray KK, Morrow DA, Gibson CM, Murphy S, Antman EM, Braunwald E. Predictors of the rise in vWF after ST elevation myocardial infarction: implications for treatment strategies and clinical outcome: an ENTIRE-TIMI 23 substudy. Eur. Heart J.26(5), 440–446 (2005).
   PubMed Web of Science ®Google Scholar
• Collet JP, Montalescot G, Vicaut E et al. Acute release of plasminogen activator inhibitor-1 in ST-segment elevation myocardial infarction predicts mortality. Circulation108(4), 391–394 (2003).
   PubMed Web of Science ®Google Scholar
• Fuchs I, Frossard M, Spiel A, Riedmuller E, Laggner AN, Jilma B. Platelet function in patients with acute coronary syndrome (ACS) predicts recurrent ACS. J. Thromb. Haemost.4(12), 2547–2552 (2006).
   PubMed Web of Science ®Google Scholar
• Jansson JH, Nilsson TK, Johnson O. Von Willebrand factor in plasma: a novel risk factor for recurrent myocardial infarction and death. Br. Heart J.66(5), 351–355 (1991).
   PubMedGoogle Scholar
• Bongers TN, de Bruijne EL, Dippel DW et al. Lower levels of ADAMTS13 are associated with cardiovascular disease in young patients. Atherosclerosis207(1), 250–254 (2009).
   PubMed Web of Science ®Google Scholar
• Crawley JT, Lane DA, Woodward M, Rumley A, Lowe GD. Evidence that high von Willebrand factor and low ADAMTS-13 levels independently increase the risk of a non-fatal heart attack. J. Thromb. Haemost.6(4), 583–588 (2008).
   PubMed Web of Science ®Google Scholar
• Kaikita K, Soejima K, Matsukawa M, Nakagaki T, Ogawa H. Reduced von Willebrand factor-cleaving protease (ADAMTS13) activity in acute myocardial infarction. J. Thromb. Haemost.4(11), 2490–2493 (2006).
   PubMed Web of Science ®Google Scholar
• Chion CK, Doggen CJ, Crawley JT, Lane DA, Rosendaal FR. ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men. Blood109(5), 1998–2000 (2007).
   PubMed Web of Science ®Google Scholar
• 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.4(10), 2103–2114 (2006).
   PubMed Web of Science ®Google Scholar
• Lillicrap D. Von Willebrand disease – phenotype versus genotype: deficiency versus disease. Thromb. Res.120(Suppl. 1), S11–S16 (2007).
   PubMed Web of Science ®Google Scholar
• Federici AB. Classification of inherited von Willebrand disease and implications in clinical practice. Thromb. Res.124(Suppl. 1), S2–S6 (2009).
   Web of Science ®Google Scholar
• Yago T, Lou J, Wu T et al. Platelet glycoprotein Ibα forms catch bonds with human WT vWF but not with type 2B von Willebrand disease vWF. J. Clin. Invest.118(9), 3195–3207 (2008).
   PubMed Web of Science ®Google Scholar
• Rayes J, Hollestelle MJ, Legendre P et al. Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B. Blood115(23), 4870–4877 (2010).
   PubMed Web of Science ®Google Scholar
• Rayes J, Hommais A, Legendre P et al. Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13. J. Thromb. Haemost.5(2), 321–328 (2007).
   PubMed Web of Science ®Google Scholar
• Jilma B, Paulinska P, Jilma-Stohlawetz P, Gilbert JC, Hutabarat R, Knobl P. A randomised pilot trial of the anti-von Willebrand factor aptamer ARC1779 in patients with type 2b von Willebrand disease. Thromb. Haemost.104 (2010).
   Google Scholar
• Casonato A, Steffan A, Pontara E et al. Post-DDAVP thrombocytopenia in type 2B von Willebrand disease is not associated with platelet consumption: failure to demonstrate glycocalicin increase or platelet activation. Thromb. Haemost.81(2), 224–228 (1999).
   PubMed Web of Science ®Google Scholar
• Favaloro EJ. Phenotypic identification of platelet-type von Willebrand disease and its discrimination from type 2B von Willebrand disease: a question of 2B or not 2B? A story of nonidentical twins? Or two sides of a multidenominational or multifaceted primary-hemostasis coin? Semin. Thromb. Hemost.34(1), 113–127 (2008).
   PubMed Web of Science ®Google Scholar
• Hillery CA, Mancuso DJ, Evan Sadler J et al. Type 2M von Willebrand disease: F606I and I662F mutations in the glycoprotein Ib binding domain selectively impair ristocetin- but not botrocetin-mediated binding of von Willebrand factor to platelets. Blood91(5), 1572–1581 (1998).
   PubMed Web of Science ®Google Scholar
• Mancuso DJ, Kroner PA, Christopherson PA, Vokac EA, Gill JC, Montgomery RR. Type 2M:Milwaukee-1 von Willebrand disease: an in-frame deletion in the Cys509–Cys695 loop of the von Willebrand factor A1 domain causes deficient binding of von Willebrand factor to platelets. Blood88(7), 2559–2568 (1996).
   PubMed Web of Science ®Google Scholar
• Rabinowitz I, Tuley EA, Mancuso DJ et al. von Willebrand disease type B: a missense mutation selectively abolishes ristocetin-induced von Willebrand factor binding to platelet glycoprotein Ib. Proc. Natl Acad. Sci. USA89(20), 9846–9849 (1992).
   PubMed Web of Science ®Google Scholar
• 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.86(3), 848–854 (2001).
   PubMed Web of Science ®Google Scholar
• Sadler JE, Matsushita T, Dong Z, Tuley EA, Westfield LA. Molecular mechanism and classification of von Willebrand disease. Thromb. Haemost.74(1), 161–166 (1995).
   PubMed Web of Science ®Google Scholar
• Aledort LM. Treatment of von Willebrand’s disease. Mayo Clin. Proc.66(8), 841–846 (1991).
   PubMed Web of Science ®Google Scholar
• Lopez JA, Andrews RK, Afshar-Kharghan V, Berndt MC. Bernard–Soulier syndrome. Blood91(12), 4397–4418 (1998).
   PubMed Web of Science ®Google Scholar
• Ware J, Russell S, Ruggeri ZM. Generation and rescue of a murine model of platelet dysfunction: the Bernard-Soulier syndrome. Proc. Natl Acad. Sci. USA97(6), 2803–2808 (2000).
   PubMed Web of Science ®Google Scholar
• Bukowski RM. Thrombotic thrombocytopenic purpura: a review. Prog. Hemost. Thromb.6:287–337 (1982).
   PubMedGoogle Scholar
• Moake JL, McPherson PD. Abnormalities of von Willebrand factor multimers in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome. Am. J. Med.87(3N), 9N–15N (1989).
   PubMed Web of Science ®Google Scholar
• Chung DW, Fujikawa K. Processing of von Willebrand factor by ADAMTS-13. Biochemistry41(37), 11065–11070 (2002).
   PubMed Web of Science ®Google Scholar
• Tasneem S, Adam F, Minullina I et al. Platelet adhesion to multimerin 1 in vitro: influences of platelet membrane receptors, von Willebrand factor and shear. J. Thromb. Haemost.7(4), 685–692 (2009).
   PubMed Web of Science ®Google Scholar
• Levy GG, Nichols WC, Lian EC et al. Mutations in a member of the ADAMTS gene family cause thrombotic thrombocytopenic purpura. Nature413(6855), 488–494 (2001).
   PubMed Web of Science ®Google Scholar
• Ruggenenti P, Noris M, Remuzzi G. Thrombotic microangiopathy, hemolytic uremic syndrome, and thrombotic thrombocytopenic purpura. Kidney Int.60(3), 831–846 (2001).
   PubMed Web of Science ®Google Scholar
• Benke S, Moltzan C. Co-existence of heparin-induced thrombocytopenia and thrombotic thrombocytopenic purpura in a postoperative cardiac surgery patient. Am. J. Hematol.80(4), 288–291 (2005).
   PubMed Web of Science ®Google Scholar
• Boctor FN, Prichard JW. Kidney involvement in thrombotic thrombocytopenic purpura and malignant hypertension. Transfusion49(9), 1783–1784 (2009).
   PubMed Web of Science ®Google Scholar
• Hughes C, McEwan JR, Longair I et al. Cardiac involvement in acute thrombotic thrombocytopenic purpura: association with troponin T and IgG antibodies to ADAMTS 13. J. Thromb. Haemost.7(4), 529–536 (2009).
   PubMed Web of Science ®Google Scholar
• Benz K, Amann K. Thrombotic microangiopathy: new insights. Curr. Opin. Nephrol. Hypertens.19(3), 242–247 (2010).
   PubMed Web of Science ®Google Scholar
• Scott EA, Puca KE, Pietz BC, Duchateau BK, Friedman KD. Comparison and stability of ADAMTS13 activity in therapeutic plasma products. Transfusion47(1), 120–125 (2007).
   PubMed Web of Science ®Google Scholar
• Peyvandi F. The role of ADAMTS13 in the new pathogenesis of TTP. Hematology10(Suppl. 1), 47–48 (2005).
   PubMed Web of Science ®Google Scholar
• Bentley MJ, Lehman CM, Blaylock RC, Wilson AR, Rodgers GM. The utility of patient characteristics in predicting severe ADAMTS13 deficiency and response to plasma exchange. Transfusion50(8), 1654–1664 (2010).
   PubMed Web of Science ®Google Scholar
• Hovinga JA, Vesely SK, Terrell DR, Lammle B, George JN. Survival and relapse in patients with thrombotic thrombocytopenic purpura. Blood115(8), 1500–1511 (2010).
   PubMed Web of Science ®Google Scholar
• Zheng XL, Kaufman RM, Goodnough LT, Sadler JE. Effect of plasma exchange on plasma ADAMTS13 metalloprotease activity, inhibitor level, and clinical outcome in patients with idiopathic and nonidiopathic thrombotic thrombocytopenic purpura. Blood103(11), 4043–4049 (2004).
   PubMed Web of Science ®Google Scholar
• Sadler JE. Von Willebrand factor, ADAMTS13, and thrombotic thrombocytopenic purpura. Blood112(1), 11–18 (2008).
   PubMed Web of Science ®Google Scholar
• Antoine G, Zimmermann K, Plaimauer B et al. ADAMTS13 gene defects in two brothers with constitutional thrombotic thrombocytopenic purpura and normalization of von Willebrand factor-cleaving protease activity by recombinant human ADAMTS13. Br. J. Haematol.120(5), 821–824 (2003).
   PubMed Web of Science ®Google Scholar
• Chauhan AK, Motto DG, Lamb CB et al. Systemic antithrombotic effects of ADAMTS13. J. Exp. Med.203(3), 767–776 (2006).
   PubMed Web of Science ®Google Scholar
• Mayr FB, Knobl P, Jilma B et al. The aptamer ARC1779 blocks von Willebrand factor-dependent platelet function in patients with thrombotic thrombocytopenic purpura ex vivo. Transfusion50(5), 1079–1087 (2010).
   PubMed Web of Science ®Google Scholar
• Jilma B, Jilma P, Paulinska P et al. Proof of concept for the anti von Willebrand factor aptamer in patients with relapsing thrombotic thrombocytopenic purpura (TTP). Blood112(11), 798–799 (2008).
   Google Scholar
• Spiel AO, Mayr FB, Ladani N et al. The aptamer ARC1779 is a potent and specific inhibitor of von Willebrand factor mediated ex vivo platelet function in acute myocardial infarction. Platelets20(5), 334–340 (2009).
   PubMed Web of Science ®Google Scholar
• Spiel AO, Siller-Matula J, Firbas C, Leitner JM, Russmueller G, Jilma B. Single dose granulocyte colony-stimulating factor markedly enhances shear-dependent platelet function in humans. Platelets21(6), 464–469 (2010).
   PubMed Web of Science ®Google Scholar
• Coller BS, Gralnick HR. Studies on the mechanism of ristocetin-induced platelet agglutination. Effects of structural modification of ristocetin and vancomycin. J. Clin. Invest.60(2), 302–312 (1977).
   PubMed Web of Science ®Google Scholar
• Matsui T, Hamako J. Structure and function of snake venom toxins interacting with human von Willebrand factor. Toxicon45(8), 1075–1087 (2005).
   PubMed Web of Science ®Google Scholar
• Jilma B. Platelet function analyzer (PFA-100): a tool to quantify congenital or acquired platelet dysfunction. J. Lab. Clin. Med.138(3), 152–163 (2001).
   PubMed Web of Science ®Google Scholar
• Frossard M, Fuchs I, Leitner JM et al. Platelet function predicts myocardial damage in patients with acute myocardial infarction. Circulation110(11), 1392–1397 (2004).
   PubMed Web of Science ®Google Scholar
• Marcucci R, Paniccia R, Antonucci E et al. Residual platelet reactivity is an independent predictor of myocardial injury in acute myocardial infarction patients on antiaggregant therapy. Thromb. Haemost.98(4), 844–851 (2007).
   PubMed Web of Science ®Google Scholar
• Jilma-Stohlawetz P, Horvath M, Eichelberger B, Koren D, Jilma B, Panzer S. Platelet function under high-shear conditions from platelet concentrates. Transfusion48(1), 129–135 (2008).
   PubMed Web of Science ®Google Scholar
• Panzer S, Eichelberger B, Koren D, Kaufmann K, Male C. Monitoring survival and function of transfused platelets in Bernard–Soulier syndrome by flow cytometry and a cone and plate(let) analyzer (Impact-R). Transfusion47(1), 103–106 (2007).
   PubMed Web of Science ®Google Scholar
• Kalb ML, Potura L, Scharbert G, Kozek-Langenecker SA. The effect of ex vivo anticoagulants on whole blood platelet aggregation. Platelets20(1), 7–11 (2009).
   PubMed Web of Science ®Google Scholar
• Toth O, Calatzis A, Penz S, Losonczy H, Siess W. Multiple electrode aggregometry: a new device to measure platelet aggregation in whole blood. Thromb. Haemost.96(6), 781–788 (2006).
   PubMed Web of Science ®Google Scholar
• Penz SM, Reininger AJ, Toth O, Deckmyn H, Brandl R, Siess W. Glycoprotein Ibα inhibition and ADP receptor antagonists, but not aspirin, reduce platelet thrombus formation in flowing blood exposed to atherosclerotic plaques. Thromb. Haemost.97(3), 435–443 (2007).
   PubMed Web of Science ®Google Scholar
• Eshtehardi P, Windecker S, Cook S et al. Dual low response to acetylsalicylic acid and clopidogrel is associated with myonecrosis and stent thrombosis after coronary stent implantation. Am. Heart J.159(5), 891–898.e1 (2010).
   PubMed Web of Science ®Google Scholar
• Sibbing D, Morath T, Braun S et al. Clopidogrel response status assessed with Multiplate point-of-care analysis and the incidence and timing of stent thrombosis over six months following coronary stenting. Thromb. Haemost.103(1), 151–159 (2010).
   PubMed Web of Science ®Google Scholar
• Siller-Matula JM, Christ G, Lang IM, Delle-Karth G, Huber K, Jilma B. Multiple electrode aggregometry predicts stent thrombosis better than the vasodilator-stimulated phosphoprotein phosphorylation assay. J. Thromb. Haemost.8(2), 351–359 (2010).
   PubMed Web of Science ®Google Scholar
• Wuster C, Steger G, Schmelzle A, Gottswinter J, Minne HW, Ziegler R. Increased incidence of euthyroid and hyperthyroid goiters independently of thyrotropin in patients with acromegaly. Horm. Metab. Res.23(3), 131–134 (1991).
   PubMed Web of Science ®Google Scholar
• Siller-Matula JM, Krumphuber J, Jilma B. Pharmacokinetic, pharmacodynamic and clinical profile of novel antiplatelet drugs targeting vascular diseases. Br. J. Pharmacol.159(3), 502–517 (2010).
   PubMed Web of Science ®Google Scholar
• Kageyama S, Yamamoto H, Nakazawa H et al. Pharmacokinetics and pharmacodynamics of AJW200, a humanized monoclonal antibody to von Willebrand factor, in monkeys. Arterioscler. Thromb. Vasc. Biol.22(1), 187–192 (2002).
   PubMed Web of Science ®Google Scholar
• Bouchard PR, Hutabarat RM, Thompson KM. Discovery and development of therapeutic aptamers. Annu. Rev. Pharmacol. Toxicol.50, 237–257 (2010).
   PubMed Web of Science ®Google Scholar
• Paulinska P, Spiel A, Jilma B. Role of von Willebrand factor in vascular disease. Hamostaseologie29(1), 32–38 (2009).
   PubMed Web of Science ®Google Scholar
• Diener JL, Daniel Lagasse HA, Duerschmied D et al. Inhibition of von Willebrand factor-mediated platelet activation and thrombosis by the anti-von Willebrand factor A1-domain aptamer ARC1779. J. Thromb. Haemost.7(7), 1155–1162 (2009).
   PubMed Web of Science ®Google Scholar
• Jilma B, Jilma P, Gilbert JC et al. Safety, pharmacokinetics and pharmacodynamics of the anti von Willebrand factor aptamer ARC1779 in patients with acute thrombotic thrombocytopenic purpura (TTP). J. Thromb. Haemost.7(Suppl. 1) (2009) (Abstract AS-WE-014).
   PubMed Web of Science ®Google Scholar
• Knobl P, Jilma B, Gilbert JC, Hutabarat RM, Wagner PG, Jilma-Stohlawetz P. Anti-von Willebrand factor aptamer ARC1779 for refractory thrombotic thrombocytopenic purpura. Transfusion49(10), 2181–2185 (2009).
   PubMed Web of Science ®Google Scholar
• Ulrichts H, Schoolmeester A, Hoefman S et al. Anti-thrombotic drug candidate ALX-0081 shows superior preclinical efficacy and safety compared with current marketed antiplatelet drugs. J. Thromb. Haemost. Suppl. (2009) (Abstract AS-Th-024).
   Google Scholar
• Holz J, Bartunek J, Barbato E, Vercruysse K, Pullan S, Heyndrickxs G. ALX-0081 a novel anti-thrombotic: first results of a multiple dose Phase I study in patients with stable angina undergoing PCI. J. Thromb. Haemost. Suppl. (2009) (Abstract PP-WE-416).
   Google Scholar
• Abd-Elaziz K, Kamphuisen PW, Lyssesns C et al. Safety, tolerability, pharmacokinetics and pharmacodynamics of anti-vWF nanobody ALX-0681 after single and multiple subcutaneous administrations to healthy volunteers. Presented at: 51st ASH Annual Meeting and Exposition. LA, USA, 5–8 December 2009 (Abstract).
   Google Scholar
• Vanhoorelbeke K, Depraetere H, Romijn RA, Huizinga EG, De Maeyer M, Deckmyn H. A consensus tetrapeptide selected by phage display adopts the conformation of a dominant discontinuous epitope of a monoclonal anti-vWF antibody that inhibits the von Willebrand factor-collagen interaction. J. Biol. Chem.278(39), 37815–37821 (2003).
   PubMed Web of Science ®Google Scholar
• Wu D, Meiring M, Kotze HF, Deckmyn H, Cauwenberghs N. Inhibition of platelet glycoprotein Ib, glycoprotein IIb/IIIa, or both by monoclonal antibodies prevents arterial thrombosis in baboons. Arterioscler. Thromb. Vasc. Biol.22(2), 323–328 (2002).
   PubMed Web of Science ®Google Scholar
• De Meyer SF, Staelens S, Badenhorst PN et al. Coronary artery in-stent stenosis persists despite inhibition of the von Willebrand factor–collagen interaction in baboons. Thromb. Haemost.98(6), 1343–1349 (2007).
   PubMed Web of Science ®Google Scholar
• Huizinga EG, Tsuji S, Romijn RA et al. Structures of glycoprotein Ibα and its complex with von Willebrand factor A1 domain. Science297(5584), 1176–1179 (2002).
   PubMed Web of Science ®Google Scholar
• Uff S, Clemetson JM, Harrison T, Clemetson KJ, Emsley J. Crystal structure of the platelet glycoprotein Ib(α) N-terminal domain reveals an unmasking mechanism for receptor activation. J. Biol. Chem.277(38), 35657–35663 (2002).
   PubMed Web of Science ®Google Scholar
• Cauwenberghs N, Vanhoorelbeke K, Vauterin S et al. Epitope mapping of inhibitory antibodies against platelet glycoprotein Ibα reveals interaction between the leucine-rich repeat N-terminal and C-terminal flanking domains of glycoprotein Iba. Blood98(3), 652–660 (2001).
   PubMed Web of Science ®Google Scholar
• Fontayne A, Vanhoorelbeke K, Pareyn I et al. Rational humanization of the powerful antithrombotic anti-GPIbα antibody: 6B4. Thromb. Haemost.96(5), 671–684 (2006).
   PubMed Web of Science ®Google Scholar
• 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 platelet thrombus formation in baboons. Blood99(10), 3623–3628 (2002).
   PubMed Web of Science ®Google Scholar
• Hennan JK, Swillo RE, Morgan GA et al. Pharmacologic inhibition of platelet vWF–GPIb α interaction prevents coronary artery thrombosis. Thromb. Haemost.95(3), 469–475 (2006).
   PubMed Web of Science ®Google Scholar
• Wadanoli M, Sako D, Shaw GD et al. The von Willebrand factor antagonist (GPG-290) prevents coronary thrombosis without prolongation of bleeding time. Thromb. Haemost.98(2), 397–405 (2007).
   PubMed Web of Science ®Google Scholar
• Yang J, Ji S, Dong N, Zhao Y, Ruan C. Engineering and characterization of a chimeric anti-platelet glycoprotein Ibα monoclonal antibody and preparation of its Fab fragment. Hybridoma (Larchmt)29(2), 125–132 (2010).
   PubMedGoogle Scholar
• Zhao BQ, Chauhan AK, Canault M et al. von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke. Blood114(15), 3329–3334 (2009).
   PubMed Web of Science ®Google Scholar
• Kleinschnitz C, De Meyer SF, Schwarz T et al. Deficiency of von Willebrand factor protects mice from ischemic stroke. Blood113(15), 3600–3603 (2009).
   PubMed Web of Science ®Google Scholar
• Chauhan AK, Kisucka J, Brill A, Walsh MT, Scheiflinger F, Wagner DD. ADAMTS13: a new link between thrombosis and inflammation. J. Exp. Med.205(9), 2065–2074 (2008).
   PubMed Web of Science ®Google Scholar
• Pendu R, Terraube V, Christophe OD et al. P-selectin glycoprotein ligand 1 and β2-integrins cooperate in the adhesion of leukocytes to von Willebrand factor. Blood108(12), 3746–3752 (2006).
   PubMed Web of Science ®Google Scholar