von Willebrand factor

Summary

Gene Symbol: von Willebrand factor
Description: von Willebrand factor
Alias: F8VWF, VWD, von Willebrand factor, coagulation factor VIII VWF
Species: human
Products:     von Willebrand factor

Top Publications

  1. VAN Schie M, VAN Loon J, De Maat M, Leebeek F. Genetic determinants of von Willebrand factor levels and activity in relation to the risk of cardiovascular disease: a review. J Thromb Haemost. 2011;9:899-908 pubmed publisher
    It is well established that high plasma von Willebrand factor (VWF) levels are associated with an increased risk of arterial thrombosis, including myocardial infarction and ischemic stroke...
  2. Zhou Y, Eng E, Nishida N, Lu C, Walz T, Springer T. A pH-regulated dimeric bouquet in the structure of von Willebrand factor. EMBO J. 2011;30:4098-111 pubmed publisher
    ..and Weibel-Palade bodies (WPBs), but not at the alkaline pH of secretion, the C-terminal ?1350 residues of von Willebrand factor (VWF) zip up into an elongated, dimeric bouquet...
  3. Bridges D, Bunn J, van Mourik J, Grau G, Preston R, Molyneux M, et al. Rapid activation of endothelial cells enables Plasmodium falciparum adhesion to platelet-decorated von Willebrand factor strings. Blood. 2010;115:1472-4 pubmed publisher
    During Plasmodium falciparum malaria infections, von Willebrand factor (VWF) levels are elevated, postmortem studies show platelets colocalized with sequestered infected erythrocytes (IEs) at brain microvascular sites, whereas in vitro ..
  4. Sadler J. von Willebrand factor assembly and secretion. J Thromb Haemost. 2009;7 Suppl 1:24-7 pubmed publisher
    During its life history, von Willebrand factor (VWF) experiences a remarkable sequence of conformational changes that are triggered by differences in pH between the endoplasmic reticulum (ER), Golgi and extracellular environments...
  5. Zanardelli S, Chion A, Groot E, Lenting P, McKinnon T, Laffan M, et al. A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF. Blood. 2009;114:2819-28 pubmed publisher
    ADAMTS13 metalloprotease regulates the multimeric size of von Willebrand factor (VWF) by cleaving the Tyr1605-Met1606 bond in the VWF A2 domain. The mechanisms of VWF recognition by ADAMTS13 have yet to be fully resolved...
  6. Auton M, Sowa K, Behymer M, Cruz M. N-terminal flanking region of A1 domain in von Willebrand factor stabilizes structure of A1A2A3 complex and modulates platelet activation under shear stress. J Biol Chem. 2012;287:14579-85 pubmed publisher
    b>von Willebrand factor (vWF) mediates platelet adhesion and thrombus formation via its interaction with the platelet receptor glycoprotein (GP)Ib?...
  7. Xiong Y, Huo Y, Chen C, Zeng H, Lu X, Wei C, et al. Vascular endothelial growth factor (VEGF) receptor-2 tyrosine 1175 signaling controls VEGF-induced von Willebrand factor release from endothelial cells via phospholipase C-gamma 1- and protein kinase A-dependent pathways. J Biol Chem. 2009;284:23217-24 pubmed publisher
    ..study, we showed that VEGF receptor-2 (VEGFR2), but not VEGFR1, is responsible for VEGF-induced release of von Willebrand factor (vWF), a major marker of WPBs...
  8. Campos M, Sun W, Yu F, Barbalic M, Tang W, Chambless L, et al. Genetic determinants of plasma von Willebrand factor antigen levels: a target gene SNP and haplotype analysis of ARIC cohort. Blood. 2011;117:5224-30 pubmed publisher
    b>von Willebrand factor (VWF) is an essential component of hemostasis and has been implicated in thrombosis. Multimer size and the amount of circulating VWF are known to impact hemostatic function...
  9. van Schie M, de Maat M, Isaacs A, van Duijn C, Deckers J, Dippel D, et al. Variation in the von Willebrand factor gene is associated with von Willebrand factor levels and with the risk for cardiovascular disease. Blood. 2011;117:1393-9 pubmed publisher
    High levels of von Willebrand factor (VWF) are associated with an increased risk for cardiovascular disease (CVD)...

More Information

Publications64

  1. Wang J, Valentijn K, de Boer H, Dirven R, van Zonneveld A, Koster A, et al. Intracellular storage and regulated secretion of von Willebrand factor in quantitative von Willebrand disease. J Biol Chem. 2011;286:24180-8 pubmed publisher
    Several missense mutations in the von Willebrand Factor (VWF) gene of von Willebrand disease (VWD) patients have been shown to cause impaired constitutive secretion and intracellular retention of VWF...
  2. Casari C, Pinotti M, Lancellotti S, Adinolfi E, Casonato A, De Cristofaro R, et al. The dominant-negative von Willebrand factor gene deletion p.P1127_C1948delinsR: molecular mechanism and modulation. Blood. 2010;116:5371-6 pubmed publisher
    ..Cellular models of severe type 2 von Willebrand disease, caused by a heterozygous deletion in the von Willebrand factor (VWF) gene, were produced to investigate the altered biosynthesis...
  3. Flood V, Gill J, Morateck P, Christopherson P, Friedman K, Haberichter S, et al. Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor. Blood. 2010;116:280-6 pubmed publisher
    The diagnosis of von Willebrand disease relies on abnormalities in specific tests of von Willebrand factor (VWF), including VWF antigen (VWF:Ag) and VWF ristocetin cofactor activity (VWF:RCo)...
  4. Campos M, Buchanan A, Yu F, Barbalic M, Xiao Y, Chambless L, et al. Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study. Blood. 2012;119:1929-34 pubmed publisher
    ..a cofactor for factor IXa in the contact coagulation pathway and circulates in a protective complex with von Willebrand factor (VWF)...
  5. Pérez Rodríguez A, García Rivero A, Lourés E, López Fernández M, Rodríguez Trillo A, Batlle J. Autosomal dominant C1149R von Willebrand disease: phenotypic findings and their implications. Haematologica. 2009;94:679-86 pubmed publisher
    Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD)...
  6. Ju L, Dong J, Cruz M, Zhu C. The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet glycoprotein Ib?. J Biol Chem. 2013;288:32289-301 pubmed publisher
    Binding of platelet glycoprotein Ib? (GPIb?) to von Willebrand factor (VWF) initiates platelet adhesion to disrupted vascular surface under arterial blood flow...
  7. Tischer A, Cruz M, Auton M. The linker between the D3 and A1 domains of vWF suppresses A1-GPIb? catch bonds by site-specific binding to the A1 domain. Protein Sci. 2013;22:1049-59 pubmed publisher
    Platelet attachment to von Willebrand factor (vWF) requires the interaction between the platelet GP1b? and exposed vWF-A1 domains...
  8. Nowak A, Canis K, Riddell A, Laffan M, McKinnon T. O-linked glycosylation of von Willebrand factor modulates the interaction with platelet receptor glycoprotein Ib under static and shear stress conditions. Blood. 2012;120:214-22 pubmed publisher
    ..These data demonstrate an important functional role of VWF OLGs under shear stress conditions. ..
  9. Lethagen S, Hillarp A, Ekholm C, Mattson E, Hallden C, Friberg B. Distribution of von Willebrand factor levels in young women with and without bleeding symptoms: influence of ABO blood group and promoter haplotypes. Thromb Haemost. 2008;99:1013-8 pubmed publisher
    The normal distribution of von Willebrand factor (VWF) levels is wide. Low levels are associated with bleeding symptoms and von Willebrand disease (VWD)...
  10. Daidone V, Cattini M, Pontara E, Sartorello F, Gallinaro L, Marotti A, et al. Microsatellite (GT)(n) repeats and SNPs in the von Willebrand factor gene promoter do not influence circulating von Willebrand factor levels under normal conditions. Thromb Haemost. 2009;101:298-304 pubmed
    b>Von Willebrand factor (VWF) levels vary considerably in normal individuals, influenced by inherited and acquired modulators...
  11. Dmitrieva N, Burg M. Secretion of von Willebrand factor by endothelial cells links sodium to hypercoagulability and thrombosis. Proc Natl Acad Sci U S A. 2014;111:6485-90 pubmed publisher
    ..plasma sodium concentration as a factor that modulates blood coagulability by affecting the production of von Willebrand factor (vWF), a key initiator of the clotting cascade...
  12. Smith N, Rice K, Bovill E, Cushman M, Bis J, McKnight B, et al. Genetic variation associated with plasma von Willebrand factor levels and the risk of incident venous thrombosis. Blood. 2011;117:6007-11 pubmed publisher
    ..Both STXBP5 and VWF findings were replicated successfully. Variation in genes associated with VWF levels in the genome-wide association study was found to be independently associated with incident VT. ..
  13. Starke R, Ferraro F, Paschalaki K, Dryden N, McKinnon T, Sutton R, et al. Endothelial von Willebrand factor regulates angiogenesis. Blood. 2011;117:1071-80 pubmed publisher
    ..VWD is caused by a defect or deficiency in von Willebrand factor (VWF), a glycoprotein essential for normal hemostasis that is involved in inflammation...
  14. Daidone V, Gallinaro L, Grazia Cattini M, Pontara E, Bertomoro A, Pagnan A, et al. An apparently silent nucleotide substitution (c.7056C>T) in the von Willebrand factor gene is responsible for type 1 von Willebrand disease. Haematologica. 2011;96:881-7 pubmed publisher
    ..We report an altered splicing process induced by a silent substitution (c.7056C>T) in the von Willebrand factor gene in a case of type 1 von Willebrand disease originally classified as lacking von Willebrand factor ..
  15. Lancellotti S, De Filippis V, Pozzi N, Oggianu L, Rutella S, Scaglione G, et al. Oxidized von Willebrand factor is efficiently cleaved by serine proteases from primary granules of leukocytes: divergence from ADAMTS-13. J Thromb Haemost. 2011;9:1620-7 pubmed publisher
    The leukocyte serine proteases (LSPs) elastase, proteinase 3 and cathepsin G cleave von Willebrand factor (VWF) near or at the same cleavage site (Tyr1605-Met1606) as ADAMTS-13, the metalloprotease that specifically controls the ..
  16. Fu X, Chen J, Gallagher R, Zheng Y, Chung D, Lopez J. Shear stress-induced unfolding of VWF accelerates oxidation of key methionine residues in the A1A2A3 region. Blood. 2011;118:5283-91 pubmed publisher
    ..These findings suggest that neutrophil oxidants will both render newly secreted VWF uncleavable and alter the largest plasma VWF forms such that they become hyperfunctional and resistant to proteolysis by ADAMTS13. ..
  17. Smith N, Chen M, Dehghan A, Strachan D, Basu S, Soranzo N, et al. Novel associations of multiple genetic loci with plasma levels of factor VII, factor VIII, and von Willebrand factor: The CHARGE (Cohorts for Heart and Aging Research in Genome Epidemiology) Consortium. Circulation. 2010;121:1382-92 pubmed publisher
    Plasma levels of coagulation factors VII (FVII), VIII (FVIII), and von Willebrand factor (vWF) influence risk of hemorrhage and thrombosis. We conducted genome-wide association studies to identify new loci associated with plasma levels...
  18. Pozzi N, Lancellotti S, De Cristofaro R, De Filippis V. Modeling ADAMTS13-von Willebrand factor interaction: Implications for oxidative stress-related cardiovascular diseases and type 2A von Willebrand disease. Biophys Chem. 2012;160:1-11 pubmed publisher
    The haemostatic potential of von Willebrand factor, a glycoprotein expressed by endothelial cells as ultra-large polymers (UL-vWF)(1), increases with its length, which in turn is regulated proteolytically by ADAMTS13, a zinc-..
  19. Ruixing Y, Jinzhen W, Shangling P, Weixiong L, Dezhai Y, Yuming C. Sex differences in environmental and genetic factors for hypertension. Am J Med. 2008;121:811-9 pubmed publisher
    ..lipase, peroxisome proliferator-activated receptor gamma, thyrotropin-releasing hormone receptor, and von Willebrand factor also was performed in these subjects...
  20. Ferlitsch M, Reiberger T, Hoke M, Salzl P, Schwengerer B, Ulbrich G, et al. von Willebrand factor as new noninvasive predictor of portal hypertension, decompensation and mortality in patients with liver cirrhosis. Hepatology. 2012;56:1439-47 pubmed publisher
    b>von Willebrand factor antigen (vWF-Ag) is elevated in patients with liver cirrhosis, but the clinical significance is unclear...
  21. Raife T, Cao W, Atkinson B, Bedell B, Montgomery R, Lentz S, et al. Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site. Blood. 2009;114:1666-74 pubmed publisher
    The function of von Willebrand factor (VWF) is regulated by proteolysis, which limits its multimeric size and ability to tether platelets...
  22. Frankel D, Meigs J, Massaro J, Wilson P, O Donnell C, D Agostino R, et al. Von Willebrand factor, type 2 diabetes mellitus, and risk of cardiovascular disease: the framingham offspring study. Circulation. 2008;118:2533-9 pubmed publisher
    b>Von Willebrand factor (vWF) is inconsistently associated with cardiovascular disease (CVD). This might be explained by associations of vWF with type 2 diabetes mellitus and insulin resistance...
  23. Zhang Q, Zhou Y, Zhang C, Zhang X, Lu C, Springer T. Structural specializations of A2, a force-sensing domain in the ultralarge vascular protein von Willebrand factor. Proc Natl Acad Sci U S A. 2009;106:9226-31 pubmed publisher
    The lengths of von Willebrand factor (VWF) concatamers correlate with hemostatic potency...
  24. Corrales I, Ramírez L, Altisent C, Parra R, Vidal F. Rapid molecular diagnosis of von Willebrand disease by direct sequencing. Detection of 12 novel putative mutations in VWF gene. Thromb Haemost. 2009;101:570-6 pubmed
    Molecular diagnosis of von Willebrand Disease (VWD) is particularly complex. The autosomal von Willebrand factor gene (VWF) is large and highly polymorphic, and there is a highly homologous (>96%) partial pseudogene in chromosome 22...
  25. Chen J, Ling M, Fu X, Lopez J, Chung D. Simultaneous exposure of sites in von Willebrand factor for glycoprotein Ib binding and ADAMTS13 cleavage: studies with ristocetin. Arterioscler Thromb Vasc Biol. 2012;32:2625-30 pubmed publisher
    Platelet-bound von Willebrand factor (VWF) was recently demonstrated to be a better substrate for ADAMTS13, suggesting that 1 conformational change exposes both the glycoprotein Ib? binding site in the A1 domain and the ADAMTS13 cleavage ..
  26. De Filippis V, Lancellotti S, Maset F, Spolaore B, Pozzi N, Gambaro G, et al. Oxidation of Met1606 in von Willebrand factor is a risk factor for thrombotic and septic complications in chronic renal failure. Biochem J. 2012;442:423-32 pubmed publisher
    ..HD patients have increased levels of vWF (von Willebrand factor), a large protein (~240 kDa) released as UL-vWF (ultra large-vWF polymers, molecular mass ~20000-50000 ..
  27. Della Morte D, Beecham A, Dong C, Wang L, McClendon M, Gardener H, et al. Association between variations in coagulation system genes and carotid plaque. J Neurol Sci. 2012;323:93-8 pubmed publisher
    ..05) with plaque size. SERPINE1 was an additional gene of interest in the haplotype and interaction analyses. Variation in the vWF, THBS1, and SERPINE1 gene may play an important role in the pathogenesis of atherosclerotic plaque. ..
  28. Bellissimo D, Christopherson P, Flood V, Gill J, Friedman K, Haberichter S, et al. VWF mutations and new sequence variations identified in healthy controls are more frequent in the African-American population. Blood. 2012;119:2135-40 pubmed publisher
    Diagnosis and classification of VWD is aided by molecular analysis of the VWF gene...
  29. JOHNSEN J, Auer P, Morrison A, Jiao S, Wei P, Haessler J, et al. Common and rare von Willebrand factor (VWF) coding variants, VWF levels, and factor VIII levels in African Americans: the NHLBI Exome Sequencing Project. Blood. 2013;122:590-7 pubmed publisher
    ..NHLBI Exome Sequencing Project, we assessed the association of these and other VWF coding variants with von Willebrand factor (VWF) and factor VIII (FVIII) levels in 4468 AAs...
  30. Skipwith C, Cao W, Zheng X. Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress. J Biol Chem. 2010;285:28596-603 pubmed publisher
    Previous studies have demonstrated that factor VIII (FVIII) or platelets alone increase cleavage of von Willebrand factor (VWF) by ADAMTS13 under mechanically induced shear stresses...
  31. VAN Schie M, Wieberdink R, Koudstaal P, Hofman A, Ikram M, Witteman J, et al. Genetic determinants of von Willebrand factor plasma levels and the risk of stroke: the Rotterdam Study. J Thromb Haemost. 2012;10:550-6 pubmed publisher
    High von Willebrand factor (VWF) plasma levels are associated with an increased risk of stroke. VWF levels are strongly heritable...
  32. Kim J, Zhang C, Zhang X, Springer T. A mechanically stabilized receptor-ligand flex-bond important in the vasculature. Nature. 2010;466:992-5 pubmed publisher
    Haemostasis in the arteriolar circulation mediated by von Willebrand factor (VWF) binding to platelets is an example of an adhesive interaction that must withstand strong hydrodynamic forces acting on cells...
  33. Cao W, Krishnaswamy S, Camire R, Lenting P, Zheng X. Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13. Proc Natl Acad Sci U S A. 2008;105:7416-21 pubmed publisher
    Proteolytic processing of von Willebrand factor (VWF) by ADAMTS13 metalloproteinase is crucial for normal hemostasis...
  34. Baronciani L, Federici A, Punzo M, Solimando M, Cozzi G, La Marca S, et al. Type 2A (IIH) von Willebrand disease is due to mutations that affect von Willebrand factor multimerization. J Thromb Haemost. 2009;7:1114-22 pubmed publisher
    ..The propositus had normal von Willebrand factor (VWF):ristocetin cofactor activity (RCo) and high VWF antigen (VWF:Ag) values, with a low VWF:RCo/VWF:Ag ..
  35. Daidone V, Pontara E, Romualdi C, Cattini M, Scaroni C, Albiger N, et al. Microsatellite (GT)(n) is part of the von Willebrand factor (VWF) promoter region that influences the glucocorticoid-induced increase in VWF in Cushing's syndrome. Thromb Res. 2010;125:e275-80 pubmed publisher
    The cortisol-induced increase in von Willebrand factor (VWF) in Cushing's syndrome (CS) seems to depend on single nucleotide polymorphisms (SNPs) of the VWF promoter, haplotype 1 (-3268G/-2709C/-2661A/-2527G) being the susceptible ..
  36. Castaman G, Giacomelli S, Jacobi P, Obser T, Budde U, Rodeghiero F, et al. Homozygous type 2N R854W von Willebrand factor is poorly secreted and causes a severe von Willebrand disease phenotype. J Thromb Haemost. 2010;8:2011-6 pubmed publisher
    ..disease (VWD) type Normandy (VWD 2N) is caused by mutations at the factor (F)VIII-binding site of von Willebrand factor (VWF), located in the D'and D3 domains on the N-terminus of mature VWF...
  37. Morange P, Saut N, Antoni G, Emmerich J, Tregouet D. Impact on venous thrombosis risk of newly discovered gene variants associated with FVIII and VWF plasma levels. J Thromb Haemost. 2011;9:229-31 pubmed publisher
  38. Dayananda K, Singh I, Mondal N, Neelamegham S. von Willebrand factor self-association on platelet GpIbalpha under hydrodynamic shear: effect on shear-induced platelet activation. Blood. 2010;116:3990-8 pubmed publisher
    The function of the mechanosensitive, multimeric blood protein von Willebrand factor (VWF) is dependent on its size...
  39. Bostan F, Coban E. The relationship between levels of von Willebrand factor and mean platelet volume in subjects with isolated impaired fasting glucose. Med Sci Monit. 2011;17:PR1-4 pubmed
    To understand the heterogeneity of platelets, we investigated the correlation between von Willebrand factor (vWf) and mean platelet volume (MPV) in subjects with isolated impaired fasting glucose (IFG)...
  40. Buzza M, Dyson J, Choi H, Gardiner E, Andrews R, Kaiserman D, et al. Antihemostatic activity of human granzyme B mediated by cleavage of von Willebrand factor. J Biol Chem. 2008;283:22498-504 pubmed publisher
    ..GrB to cleave extracellular matrix proteins, we examined the effect of GrB on the pro-hemostatic molecule von Willebrand factor (VWF)...
  41. Lanke E, Kristoffersson A, Philips M, Holmberg L, Lethagen S. Characterization of a novel mutation in the von Willebrand factor propeptide in a distinct subtype of recessive von Willebrand disease. Thromb Haemost. 2008;100:211-6 pubmed
    b>von Willebrand factor (VWF) is a plasma protein that consists of a series of multimers of which the high-molecular-weight VWF multimers are the most potent in platelet adhesion and aggregation...
  42. Yago T, Lou J, Wu T, Yang J, Miner J, Coburn L, et al. Platelet glycoprotein Ibalpha forms catch bonds with human WT vWF but not with type 2B von Willebrand disease vWF. J Clin Invest. 2008;118:3195-207 pubmed publisher
    ..Mutations in the vWF A1 domain that cause type 2B von Willebrand disease (vWD) reduce the flow requirement for adhesion...
  43. Huang J, Roth R, Heuser J, Sadler J. Integrin alpha(v)beta(3) on human endothelial cells binds von Willebrand factor strings under fluid shear stress. Blood. 2009;113:1589-97 pubmed publisher
    Acutely secreted von Willebrand factor (VWF) multimers adhere to endothelial cells, support platelet adhesion, and may induce microvascular thrombosis...
  44. Zhou Y, Eng E, Zhu J, Lu C, Walz T, Springer T. Sequence and structure relationships within von Willebrand factor. Blood. 2012;120:449-58 pubmed publisher
    In the present study, we re-annotated von Willebrand factor (VWF), assigned its entire sequence to specific modules, and related these modules to structure using electron microscopy (EM)...
  45. Madabhushi S, Shang C, Dayananda K, Rittenhouse Olson K, Murphy M, Ryan T, et al. von Willebrand factor (VWF) propeptide binding to VWF D'D3 domain attenuates platelet activation and adhesion. Blood. 2012;119:4769-78 pubmed publisher
    Noncovalent association between the von Willebrand factor (VWF) propeptide (VWFpp) and mature VWF aids N-terminal multimerization and protein compartmentalization in storage granules...
  46. Singh I, Themistou E, Porcar L, Neelamegham S. Fluid shear induces conformation change in human blood protein von Willebrand factor in solution. Biophys J. 2009;96:2313-20 pubmed publisher
    Many of the physiological functions of von Willebrand Factor (VWF), including its binding interaction with blood platelets, are regulated by the magnitude of applied fluid/hydrodynamic stress...
  47. Zhang X, Halvorsen K, Zhang C, Wong W, Springer T. Mechanoenzymatic cleavage of the ultralarge vascular protein von Willebrand factor. Science. 2009;324:1330-4 pubmed publisher
    b>Von Willebrand factor (VWF) is secreted as ultralarge multimers that are cleaved in the A2 domain by the metalloprotease ADAMTS13 to give smaller multimers...
  48. Chen J, Fu X, Wang Y, Ling M, McMullen B, Kulman J, et al. Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13. Blood. 2010;115:706-12 pubmed publisher
    Elevated plasma von Willebrand factor (VWF) and low ADAMTS13 activity have been reported in several inflammatory states, including sepsis and acute respiratory distress syndrome...
  49. Tanka Salamon A, Kolev K, Machovich R, Komorowicz E. Proteolytic resistance conferred to fibrinogen by von Willebrand factor. Thromb Haemost. 2010;103:291-8 pubmed publisher
    The formation of platelet-rich thrombi under high shear rates requires both fibrinogen and von Willebrand factor (VWF) as molecular adhesives between platelets...
  50. Schneppenheim R, Michiels J, Obser T, Oyen F, Pieconka A, Schneppenheim S, et al. A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE. Blood. 2010;115:4894-901 pubmed publisher
    ..a particular subgroup with a high frequency of 29%, characterized by a relative decrease of large von Willebrand factor (VWF) multimers and decreased A Disintegrin And Metalloproteinase with ThromboSpondin type 1 motifs, ..
  51. Xia Z, Yang H, Qu H, Cheng W, Wang L. Expression of P-selectin, von Willebrand and endothelin-1 after carotid artery stenting. Vasa. 2011;40:199-204 pubmed publisher
    ..The post-CAS levels of P-selectin, vWF and endothelin-1 are related to the extent of endothelial injury. Whether they are associated with restenosis 12 months after the treatment requires further investigation. ..
  52. Spiel A, Gilbert J, Jilma B. von Willebrand factor in cardiovascular disease: focus on acute coronary syndromes. Circulation. 2008;117:1449-59 pubmed publisher
    b>von Willebrand factor (VWF) plays a pivotal role in platelet adhesion and aggregation at sites of high shear rates (eg, in coronary arteries that have stenotic or ruptured atherosclerotic plaque lesions)...
  53. Singh I, Shankaran H, Beauharnois M, Xiao Z, Alexandridis P, Neelamegham S. Solution structure of human von Willebrand factor studied using small angle neutron scattering. J Biol Chem. 2006;281:38266-75 pubmed
    b>von Willebrand factor (VWF) binding to platelets under high fluid shear is an important step regulating atherothrombosis...
  54. Ruggeri Z. Von Willebrand factor. Curr Opin Hematol. 2003;10:142-9 pubmed
    The adhesive protein von Willebrand factor contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury...
  55. Dumas J, Kumar R, McDonagh T, Sullivan F, Stahl M, Somers W, et al. Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ibalpha complex reveals conformation differences with a complex bearing von Willebrand disease mutations. J Biol Chem. 2004;279:23327-34 pubmed
    ..direct interaction between the platelet receptor glycoprotein Ibalpha (GpIbalpha) and the A1 domain of the von Willebrand factor (VWF). Here we report the 2...