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

Top Publications

  1. ncbi Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis
    Wolf Achim Hassenpflug
    Dept of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Martinistrasse 52, D 20246 Hamburg, Germany
    Blood 107:2339-45. 2006
  2. doi Genetic determinants of von Willebrand factor levels and activity in relation to the risk of cardiovascular disease: a review
    M C VAN Schie
    Department of Haematology, Erasmus University Medical Centre, Rotterdam, The Netherlands
    J Thromb Haemost 9:899-908. 2011
  3. pmc A pH-regulated dimeric bouquet in the structure of von Willebrand factor
    Yan Feng Zhou
    Department of Pathology, Harvard Medical School, Immune Disease Institute, Program in Cellular and Molecular Medicine, Children s Hospital, Boston, MA 02115, USA
    EMBO J 30:4098-111. 2011
  4. ncbi von Willebrand factor A1 domain can adequately substitute for A3 domain in recruitment of flowing platelets to collagen
    A Bonnefoy
    Center for Molecular and Vascular Biology, University of Leuven, Leuven, Belgium
    J Thromb Haemost 4:2151-61. 2006
  5. pmc Sequence and structure relationships within von Willebrand factor
    Yan Feng Zhou
    Department of Biological Chemistry and Molecular Pharmacology, Immune Disease Institute and Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Blood 120:449-58. 2012
  6. pmc Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study
    Marco Campos
    Cardiovascular Sciences Section, Department of Medicine, Baylor College of Medicine, Houston, TX, USA
    Blood 119:1929-34. 2012
  7. pmc Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13
    Junmei Chen
    Research Division, Puget Sound Blood Center, Seattle, WA, USA
    Blood 115:706-12. 2010
  8. ncbi Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ibalpha complex reveals conformation differences with a complex bearing von Willebrand disease mutations
    John J Dumas
    Department of Chemical and Screening Sciences, Wyeth, Cambridge, Massachusetts 02140, USA
    J Biol Chem 279:23327-34. 2004
  9. doi Basal secretion of von Willebrand factor from human endothelial cells
    Jonathan P Giblin
    Department of Molecular Neuroendocrinology, Medical Research Council National Institute for Medical Research, London, United Kingdom
    Blood 112:957-64. 2008
  10. pmc Genetic variation associated with plasma von Willebrand factor levels and the risk of incident venous thrombosis
    Nicholas L Smith
    Department of Epidemiology, Cardiovascular Health Research Unit, University of Washington, 1730 Minor Avenue, Seattle, WA 98101, USA
    Blood 117:6007-11. 2011

Research Grants

  1. Von Willebrand Factor Multimer Assembly and Structure
    J Evan Sadler; Fiscal Year: 2012
  2. Veronica H Flood; Fiscal Year: 2014
  3. David Ginsburg; Fiscal Year: 2016
  4. Prevention of the Complications of Bleeding Disorders Through HTCS
    Regina Butler; Fiscal Year: 2010
  5. TRANSGENIC ANALYSIS OF PLATELET RECEPTOR EXPRESSION
    Jerry Ware; Fiscal Year: 2012
  6. Michael R King; Fiscal Year: 2014
  7. Timothy A Springer; Fiscal Year: 2015
  8. BIOMECHANICS OF UNFOLDING THE A2 DOMAIN OF VON WILLEBRAND FACTOR
    Jin Yu Shao; Fiscal Year: 2012
  9. Structural bases of ADAMTS-13 and VWF A2 interactions
    Cheng Zhu; Fiscal Year: 2013
  10. CHAR MARIE WITMER; Fiscal Year: 2014

Detail Information

Publications265 found, 100 shown here

  1. ncbi Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis
    Wolf Achim Hassenpflug
    Dept of Pediatric Hematology and Oncology, University Medical Center Hamburg Eppendorf, Martinistrasse 52, D 20246 Hamburg, Germany
    Blood 107:2339-45. 2006
    ..the most common qualitative defect of VWD, is caused by loss of high-molecular-weight multimers (HMWMs) of von Willebrand factor (VWF). Underlying mutations cluster in the A2 domain of VWF around its cleavage site for ADAMTS13...
  2. doi Genetic determinants of von Willebrand factor levels and activity in relation to the risk of cardiovascular disease: a review
    M C VAN Schie
    Department of Haematology, Erasmus University Medical Centre, Rotterdam, The Netherlands
    J Thromb Haemost 9:899-908. 2011
    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...
  3. pmc A pH-regulated dimeric bouquet in the structure of von Willebrand factor
    Yan Feng Zhou
    Department of Pathology, Harvard Medical School, Immune Disease Institute, Program in Cellular and Molecular Medicine, Children s Hospital, Boston, MA 02115, USA
    EMBO J 30:4098-111. 2011
    ..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...
  4. ncbi von Willebrand factor A1 domain can adequately substitute for A3 domain in recruitment of flowing platelets to collagen
    A Bonnefoy
    Center for Molecular and Vascular Biology, University of Leuven, Leuven, Belgium
    J Thromb Haemost 4:2151-61. 2006
    Binding of von Willebrand factor (VWF) to platelet GPIbalpha and to collagen is attributed to VWF A1 and A3 domains, respectively.
  5. pmc Sequence and structure relationships within von Willebrand factor
    Yan Feng Zhou
    Department of Biological Chemistry and Molecular Pharmacology, Immune Disease Institute and Children s Hospital, Harvard Medical School, Boston, MA 02115, USA
    Blood 120:449-58. 2012
    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)...
  6. pmc Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study
    Marco Campos
    Cardiovascular Sciences Section, Department of Medicine, Baylor College of Medicine, Houston, TX, USA
    Blood 119:1929-34. 2012
    ..a cofactor for factor IXa in the contact coagulation pathway and circulates in a protective complex with von Willebrand factor (VWF)...
  7. pmc Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13
    Junmei Chen
    Research Division, Puget Sound Blood Center, Seattle, WA, USA
    Blood 115:706-12. 2010
    Elevated plasma von Willebrand factor (VWF) and low ADAMTS13 activity have been reported in several inflammatory states, including sepsis and acute respiratory distress syndrome...
  8. ncbi Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ibalpha complex reveals conformation differences with a complex bearing von Willebrand disease mutations
    John J Dumas
    Department of Chemical and Screening Sciences, Wyeth, Cambridge, Massachusetts 02140, USA
    J Biol Chem 279:23327-34. 2004
    ..direct interaction between the platelet receptor glycoprotein Ibalpha (GpIbalpha) and the A1 domain of the von Willebrand factor (VWF). Here we report the 2...
  9. doi Basal secretion of von Willebrand factor from human endothelial cells
    Jonathan P Giblin
    Department of Molecular Neuroendocrinology, Medical Research Council National Institute for Medical Research, London, United Kingdom
    Blood 112:957-64. 2008
    Endothelial cells store the adhesive glycoprotein von Willebrand factor (VWF) in Weibel-Palade bodies (WPBs), distinctively shaped regulated secretory organelles that undergo exocytosis in response to secretagogue...
  10. pmc Genetic variation associated with plasma von Willebrand factor levels and the risk of incident venous thrombosis
    Nicholas L Smith
    Department of Epidemiology, Cardiovascular Health Research Unit, University of Washington, 1730 Minor Avenue, Seattle, WA 98101, USA
    Blood 117:6007-11. 2011
    ..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...
  11. ncbi Physical stress as a model to study variations in ADAMTS-13 activity, von Willebrand factor level and platelet activation
    R A Claus
    J Thromb Haemost 4:902-5. 2006
  12. doi Distribution of von Willebrand factor levels in young women with and without bleeding symptoms: influence of ABO blood group and promoter haplotypes
    Stefan Lethagen
    Department for Coagulation Disorders, Malmo University Hospital, Sweden
    Thromb Haemost 99:1013-8. 2008
    The normal distribution of von Willebrand factor (VWF) levels is wide. Low levels are associated with bleeding symptoms and von Willebrand disease (VWD)...
  13. pmc Fluid shear induces conformation change in human blood protein von Willebrand factor in solution
    Indrajeet Singh
    Chemical and Biological Engineering, State University of New York, Buffalo, New York, USA
    Biophys J 96:2313-20. 2009
    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...
  14. ncbi The -1185 A/G and -1051 G/A dimorphisms in the von Willebrand factor gene promoter and risk of myocardial infarction
    R Di Bitondo
    Division of Genomic Medicine, Royal Hallamshire Hospital, University of Sheffield, UK
    Br J Haematol 115:701-6. 2001
    Elevated plasma von Willebrand factor (VWF) levels are associated with coronary artery disease, although the precise mechanism for this is unclear...
  15. ncbi Microsatellite (GT)(n) repeats and SNPs in the von Willebrand factor gene promoter do not influence circulating von Willebrand factor levels under normal conditions
    Viviana Daidone
    Department of Medical and Surgical Sciences, Via Ospedale Civile 105, Padova, Italy
    Thromb Haemost 101:298-304. 2009
    b>Von Willebrand factor (VWF) levels vary considerably in normal individuals, influenced by inherited and acquired modulators...
  16. ncbi Inducible secretion of large, biologically potent von Willebrand factor multimers
    L A Sporn
    Cell 46:185-90. 1986
    b>von Willebrand factor (vWf) secreted constitutively by human endothelial cells was compared to that released from Weibel-Palade bodies after stimulation...
  17. ncbi High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability
    Tamara N Bongers
    Department of Hematology, Erasmus University Medical Center, Rotterdam, The Netherlands
    Stroke 37:2672-7. 2006
    Elevated von Willebrand factor (vWF) concentrations are associated with an increased risk of ischemic heart disease...
  18. pmc Genetic determinants of plasma von Willebrand factor antigen levels: a target gene SNP and haplotype analysis of ARIC cohort
    Marco Campos
    Section of Cardiology, Baylor College of Medicine, Houston, TX, USA
    Blood 117:5224-30. 2011
    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...
  19. doi Expression of P-selectin, von Willebrand and endothelin-1 after carotid artery stenting
    Z Y Xia
    Department of Neurology, Liaocheng People s Hospital and Liaocheng Clinical School of Taishan Medical University, Liaocheng, Shandong, PR China
    Vasa 40:199-204. 2011
    ..This study was designed to investigate the impact of carotid artery stenting (CAS) on plasma levels of P-selectin, von Willebrand (vWF) and endothelin-1...
  20. ncbi Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease-causing variants of von Willebrand factor
    Gregoire Michaux
    Medical Research Council, Laboratory for Molecular Cell Biology, University College London, United Kingdom
    Blood 102:2452-8. 2003
    The rapid exocytosis of von Willebrand factor (VWF) in response to vascular injury can be attributed to the fact that VWF is stored in the Weibel-Palade bodies (WPBs) of endothelial cells...
  21. doi Identification of a novel 14-3-3zeta binding site within the cytoplasmic domain of platelet glycoprotein Ibalpha that plays a key role in regulating the von Willebrand factor binding function of glycoprotein Ib-IX
    Yanhong Yuan
    School of Biological Science and Medical Engineering, Beijing University of Aeronautics and Astronautics, 37 Xueyuan Road, Haidian District, Beijing, China
    Circ Res 105:1177-85. 2009
    The interaction between platelet glycoprotein (GP) Ib-IX and von Willebrand factor (VWF) is initiated by conformational changes in immobilized VWF and is also regulated by the intraplatelet proteins 14-3-3zeta and filamin A...
  22. doi Oxidized von Willebrand factor is efficiently cleaved by serine proteases from primary granules of leukocytes: divergence from ADAMTS-13
    S Lancellotti
    Institute of Internal Medicine and Geriatrics, and Hemostasis Research Center, Catholic University School of Medicine, Rome, Italy
    J Thromb Haemost 9:1620-7. 2011
    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 ..
  23. ncbi Interaction between von Willebrand factor and glycoprotein Ib activates Src kinase in human platelets: role of phosphoinositide 3-kinase
    Yi Wu
    Department of Clinical and Laboratory Medicine, Faculty of Medicine, University of Yamanashi, Tamaho, Nakakoma, Yamanashi, Japan
    Blood 101:3469-76. 2003
    The binding of von Willebrand factor (VWF) to glycoprotein (GP) Ib-IX-V stimulates transmembrane signaling events that lead to platelet adhesion and aggregation...
  24. ncbi Rapid molecular diagnosis of von Willebrand disease by direct sequencing. Detection of 12 novel putative mutations in VWF gene
    Irene Corrales
    Unitat de Diagnòstic i Teràpia Molecular, Banc de Sang i Teixits, Passeig Vall d Hebron 119 129, 08035 Barcelona, Spain
    Thromb Haemost 101:570-6. 2009
    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. doi Nitric oxide inhibits von Willebrand factor-mediated platelet adhesion and spreading through regulation of integrin alpha(IIb)beta(3) and myosin light chain
    W Roberts
    Hull York Medical School, University of Hull, Castle Hill Hospital, Cottingham, UK
    J Thromb Haemost 7:2106-15. 2009
    b>von Willebrand factor (VWF)-mediated platelet adhesion and spreading at sites of vascular injury is a critical step in hemostasis. This process requires two individual receptors: glycoprotein Ib (GPIb)-V-IX and integrin alpha(IIb)beta(3)...
  26. pmc Shear stress-induced unfolding of VWF accelerates oxidation of key methionine residues in the A1A2A3 region
    Xiaoyun Fu
    Research Division, Puget Sound Blood Center, Seattle, WA 98104, USA
    Blood 118:5283-91. 2011
    ..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...
  27. doi Oxidation of Met1606 in von Willebrand factor is a risk factor for thrombotic and septic complications in chronic renal failure
    Vincenzo De Filippis
    Laboratory of Protein Chemistry, Department of Pharmaceutical Sciences, University of Padova, Padua, Italy
    Biochem J 442:423-32. 2012
    ..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 ..
  28. pmc Endothelial von Willebrand factor regulates angiogenesis
    Richard D Starke
    Cardiovascular Sciences, National Heart and Lung Institute, Faculty of Medicine, Hammersmith Campus, Imperial College Academic Health Sciences Centre, Imperial College London, London, UK
    Blood 117:1071-80. 2011
    ..VWD is caused by a defect or deficiency in von Willebrand factor (VWF), a glycoprotein essential for normal hemostasis that is involved in inflammation...
  29. ncbi Mapping the glycoprotein Ib-binding site in the von willebrand factor A1 domain
    M A Cruz
    Hematology Division, Department of Medicine, Brigham and Women s Hospital and Harvard Medical School, Boston, Massachusetts 02115, USA
    J Biol Chem 275:19098-105. 2000
    The von Willebrand factor (vWF) mediates platelet adhesion to exposed subendothelium at sites of vascular injury. It does this by forming a bridge between subendothelial collagen and the platelet glycoprotein Ib-IX-V complex (GPIb)...
  30. ncbi A role for p38 MAP kinase in platelet activation by von Willebrand factor
    Ilaria Canobbio
    Center of Excellence on Applied Biology, Department of Biochemistry, University of Pavia, via Bassi 21 27100 Pavia, Italy
    Thromb Haemost 91:102-10. 2004
    Platelet activation induced by von Willebrand factor (VWF) binding to the membrane GPIb-IX-V receptor involves multiple signal transduction pathways...
  31. ncbi P-selectin binds to the D'-D3 domains of von Willebrand factor in Weibel-Palade bodies
    Gregoire Michaux
    MRC Laboratory for Molecular Cell Biology, Cell Biology Unit and Department of Biochemistry, University College London, Gower Street, London WC1E 6BT, United Kingdom
    Blood 107:3922-4. 2006
    It has recently been shown that the ultralarge platelet-recruiting von Willebrand factor (VWF) strings formed immediately at exocytosis from endothelial cells may be anchored to the cell surface by interaction with the integral membrane ..
  32. doi von Willebrand factor in cardiovascular disease: focus on acute coronary syndromes
    Alexander O Spiel
    Department of Clinical Pharmacology, Medical University of Vienna, Wahringer Gurtel 18 20, 1090 Vienna, Austria
    Circulation 117:1449-59. 2008
    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)...
  33. pmc Integrin alpha(v)beta(3) on human endothelial cells binds von Willebrand factor strings under fluid shear stress
    Jing Huang
    Department of Medicine, Biochemistry and Molecular Biophysics, and Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 113:1589-97. 2009
    Acutely secreted von Willebrand factor (VWF) multimers adhere to endothelial cells, support platelet adhesion, and may induce microvascular thrombosis...
  34. pmc Structural specializations of A2, a force-sensing domain in the ultralarge vascular protein von Willebrand factor
    Qing Zhang
    Immune Disease Institute, Children s Hospital Boston and Department of Pathology, Harvard Medical School, Boston, MA 02115, USA
    Proc Natl Acad Sci U S A 106:9226-31. 2009
    The lengths of von Willebrand factor (VWF) concatamers correlate with hemostatic potency...
  35. doi von Willebrand factor assembly and secretion
    J E Sadler
    Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    J Thromb Haemost 7:24-7. 2009
    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...
  36. pmc 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
    Nicholas L Smith
    Department of Epidemiology, University of Washington, Seattle, Wash 98101, USA
    Circulation 121:1382-92. 2010
    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.
  37. doi Variation in the von Willebrand factor gene is associated with von Willebrand factor levels and with the risk for cardiovascular disease
    Marianne C van Schie
    Department of Hematology, Erasmus University Medical Centre, Rotterdam, The Netherlands
    Blood 117:1393-9. 2011
    High levels of von Willebrand factor (VWF) are associated with an increased risk for cardiovascular disease (CVD)...
  38. pmc Intracellular storage and regulated secretion of von Willebrand factor in quantitative von Willebrand disease
    Jiong Wei Wang
    Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, 2333ZA Leiden, The Netherlands
    J Biol Chem 286:24180-8. 2011
    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...
  39. pmc Regulation of the pp72syk protein tyrosine kinase by platelet integrin alpha IIb beta 3
    J Gao
    Department of Vascular Biology, Scripps Research Institute, La Jolla, CA 92037, USA
    EMBO J 16:6414-25. 1997
    ..Furthermore, there are fundamental differences in the activation of Syk by alpha IIb beta 3 and immune response receptors, suggesting a unique role for integrins in Syk function...
  40. ncbi Von Willebrand factor
    Zaverio M Ruggeri
    Room Research Laboratory for Arteriosclerosis and Thrombosis, Division of Experimental Hemostasis and Thrombosis, Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, California 92037, USA
    Curr Opin Hematol 10:142-9. 2003
    The adhesive protein von Willebrand factor contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury...
  41. pmc Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site
    Thomas J Raife
    Department of Pathology, University of Iowa Carver College of Medicine, Iowa City, IA 52240, USA
    Blood 114:1666-74. 2009
    The function of von Willebrand factor (VWF) is regulated by proteolysis, which limits its multimeric size and ability to tether platelets...
  42. ncbi Inter-relationships of indices of endothelial damage/dysfunction [circulating endothelial cells, von Willebrand factor and flow-mediated dilatation] to tissue factor and interleukin-6 in acute coronary syndromes
    Kaeng W Lee
    Haemostasis Thrombosis and Vascular Biology Unit, University Department of Medicine, City Hospital, Birmingham B18 7QH, UK
    Int J Cardiol 111:302-8. 2006
    ..CECs can be related to impaired flow-mediated vasodilatation (FMD, an index of endothelial dysfunction) and elevated plasma von Willebrand factor (vWf, also marking endothelial damage/dysfunction), TF and IL-6 in patients with ACS.
  43. ncbi Induction of specific storage organelles by von Willebrand factor propolypeptide
    D D Wagner
    New England Medical Center, Boston, Massachusetts
    Cell 64:403-13. 1991
    Endothelial cells store the multimeric adhesive glycoprotein von Willebrand factor (vWf), which promotes the formation of a platelet plug at the site of vessel injury...
  44. pmc Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factor
    D D Wagner
    J Cell Biol 102:1320-4. 1986
    Two conditions were identified that interfered with the complex polymerization process in biosynthesis of von Willebrand factor (vWf)...
  45. ncbi Amino acid sequence of human von Willebrand factor
    K Titani
    Biochemistry 25:3171-84. 1986
    The complete amino acid sequence of human von Willebrand factor (vWF) is presented. Most of the sequence was determined by analysis of the S-carboxymethylated protein...
  46. ncbi Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor
    B Savage
    Roon Research Center for Arteriosclerosis and Thrombosis, Department of Molecular and Experimental Medicine, Scripps Research Institute, La Jolla, La Jolla, California 92037, USA
    Cell 84:289-97. 1996
    ..In contrast, glycoprotein Ib alpha binding to immobilized von Willebrand factor (vWF) appears to have fast association and dissociation rates as well as high resistance to tensile stress,..
  47. ncbi Shear-dependent changes in the three-dimensional structure of human von Willebrand factor
    C A Siedlecki
    Department of Biomedical Engineering, Case Western Reserve University, Cleveland, OH 44106, USA
    Blood 88:2939-50. 1996
    The three-dimensional tertiary structure of human von Willebrand Factor (vWF) on a hydrophobic surface under aqueous conditions and different shear stress regimes was studied by atomic force microscopy (AFM)...
  48. ncbi Biochemistry and genetics of von Willebrand factor
    J E Sadler
    Howard Hughes Medical Institute, Department of Medicine, Washington University School of Medicine, Saint Louis, Missouri 63110, USA
    Annu Rev Biochem 67:395-424. 1998
    b>Von Willebrand factor (VWF) is a blood glycoprotein that is required for normal hemostasis, and deficiency of VWF, or von Willebrand disease (VWD), is the most common inherited bleeding disorder...
  49. ncbi Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb. Importance of structural integrity of the regions flanked by the CYS1272-CYS1458 disulfide bond
    Takayuki Nakayama
    First Department of Internal Medicine, Nagoya University School of Medicine, Japan
    J Biol Chem 277:22063-72. 2002
    In vitro platelet glycoprotein Ib (GPIb) binding of the human von Willebrand factor (VWF) increases markedly by exogenous modulators such as ristocetin or botrocetin, and the binding does not occur in normal circulation...
  50. ncbi Structures of glycoprotein Ibalpha and its complex with von Willebrand factor A1 domain
    Eric G Huizinga
    Department of Crystal and Structural Chemistry, Bijvoet Center for Biomolecular Research, Utrecht University, Padualaan 8, 3584 CH Utrecht, Netherlands
    Science 297:1176-9. 2002
    Transient interactions of platelet-receptor glycoprotein Ibalpha (GpIbalpha) and the plasma protein von Willebrand factor (VWF) reduce platelet velocity at sites of vascular damage and play a role in haemostasis and thrombosis...
  51. ncbi Aspects of hydrodynamic shear regulating shear-induced platelet activation and self-association of von Willebrand factor in suspension
    Harish Shankaran
    Bioengineering Laboratory, Department of Chemical Engineering, State University of New York at Buffalo, NY 14260, USA
    Blood 101:2637-45. 2003
    The binding of plasma von Willebrand factor (VWF) to platelet receptor GpIb under high hydrodynamic shear leads to platelet activation and subsequent shear-induced platelet aggregation (SIPA)...
  52. ncbi Genome-wide linkage analysis of von Willebrand factor plasma levels: results from the GAIT project
    Juan Carlos Souto
    Unitat d Hemostasia i Trombosi, Hospital de la Santa Creu i Sant Pau, Barcelona Spain
    Thromb Haemost 89:468-74. 2003
    High plasma levels of von Willebrand factor (vWF) have been associated with the risk of thromboembolic disease. As a complex trait, this phenotype must be influenced by genetic and environmental factors...
  53. ncbi VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13
    Koichi Kokame
    National Cardiovascular Center Research Institute, 5 7 1 Fujishirodai, Suita, Osaka 565 8565, Japan
    Blood 103:607-12. 2004
    ADAMTS-13 was recently identified as a new hemostatic factor, von Willebrand factor (VWF)-cleaving protease. Either congenital or acquired defects of the enzymatic activity lead to thrombotic thrombocytopenic purpura (TTP)...
  54. ncbi Genetic variability of von Willebrand factor and risk of coronary heart disease: the Rotterdam Study
    Irene M van der Meer
    Department of Epidemiology and Biostatistics, Erasmus MC, University Medical Centre, PO Box 1738, 3000 DR Rotterdam, The Netherlands
    Br J Haematol 124:343-7. 2004
    The von Willebrand factor (VWF) may be causally associated with coronary heart disease (CHD) or merely be a marker of endothelial damage...
  55. pmc Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13
    Kenji Nishio
    Howard Hughes Medical Institute and Department of Medicine, Washington University School of Medicine, St Louis, MO 63110, USA
    Proc Natl Acad Sci U S A 101:10578-83. 2004
    b>von Willebrand factor (vWF) is a multimeric plasma glycoprotein with three tandem A domains. Domains A1 and A3 bind to platelet glycoprotein Ibalpha (GPIbalpha) and collagen, respectively...
  56. ncbi Molecular modeling of the von Willebrand factor A2 Domain and the effects of associated type 2A von Willebrand disease mutations
    Jeffrey J Sutherland
    Department of Chemistry, Queen s University, K7L 3N6, Kingston, Ontario, Canada
    J Mol Model 10:259-70. 2004
    A homology model for the A2 domain of von Willebrand factor (VWF) is presented...
  57. ncbi Differential kinetics of cell surface loss of von Willebrand factor and its propolypeptide after secretion from Weibel-Palade bodies in living human endothelial cells
    Matthew J Hannah
    National Institute for Medical Research, Mill Hill, London, UK
    J Biol Chem 280:22827-30. 2005
    The time course for cell surface loss of von Willebrand factor (VWF) and the propolypeptide of VWF (proregion) following exocytosis of individual Weibel-Palade bodies (WPBs) from single human endothelial cells was analyzed...
  58. ncbi Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions
    J F Dong
    Thrombosis Research Section, Department of Medicine, Baylor College of Medicine, Houston, TX 77030, USA
    J Thromb Haemost 3:1710-6. 2005
    b>von Willebrand factor (VWF) is a critical ligand for platelet adhesion and aggregation. It is synthesized and released as multimers composed of various numbers of monomers...
  59. pmc Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions
    Zhenyin Tao
    Thrombosis Research Section, Department of Medicine, BCM286, N1319, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030, USA
    Blood 106:4139-45. 2005
    ..and metalloprotease with thrombospondin motif) converts the hyperreactive unusually large (UL) forms of von Willebrand factor (VWF) that are newly released from endothelial cells into less active plasma forms by cleaving a peptide ..
  60. ncbi Shielding of the A1 domain by the D'D3 domains of von Willebrand factor modulates its interaction with platelet glycoprotein Ib-IX-V
    Hans Ulrichts
    Laboratory for Thrombosis Research, Interdisciplinary Research Center, Katholieke Universiteit Leuven Campus Kortrijk, B 8500 Kortrijk, Belgium
    J Biol Chem 281:4699-707. 2006
    Soluble von Willebrand factor (VWF) has a low affinity for platelet glycoprotein (GP) Ibalpha and needs immobilization and/or high shear stress to enable binding of its A1 domain to the receptor...
  61. ncbi Solution structure of human von Willebrand factor studied using small angle neutron scattering
    Indrajeet Singh
    Department of Chemical and Biological Engineering, State University of New York, Buffalo, NY 14260, USA
    J Biol Chem 281:38266-75. 2006
    b>von Willebrand factor (VWF) binding to platelets under high fluid shear is an important step regulating atherothrombosis...
  62. ncbi ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men
    Chan K N K Chion
    Department of Haematology, Imperial College London, Hammersmith Hospital Campus, London, United Kingdom
    Blood 109:1998-2000. 2007
    b>Von Willebrand factor (VWF) mediates the tethering/adhesion of platelets at sites of vascular injury. This function depends on its multimeric size, which is controlled by ADAMTS13...
  63. ncbi Variations in glycosylation of von Willebrand factor with O-linked sialylated T antigen are associated with its plasma levels
    Carina J M van Schooten
    Laboratory for Thrombosis and Haemostasis, Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, Utrecht, The Netherlands
    Blood 109:2430-7. 2007
    The glycosylation profile of von Willebrand factor (VWF) is known to strongly influence its plasma levels...
  64. ncbi The presence of active von Willebrand factor under various pathological conditions
    Evelyn Groot
    Laboratory for Thrombosis and Haemostasis, Department of Clinical Chemistry and Haematology, University Medical Center Utrecht, The Netherlands
    Curr Opin Hematol 14:284-9. 2007
    To highlight mechanisms that regulate the balance between latent and active von Willebrand factor (VWF), and describe pathological conditions leading to increased levels of active VWF.
  65. pmc Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress
    Kyuhwan Shim
    Department of Medicine, Howard Hughes Medical Institute, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 111:651-7. 2008
    Endothelial cells secrete prothrombotic ultralarge von Willebrand factor (VWF) multimers, and the metalloprotease ADAMTS13 cleaves them into smaller, less dangerous multimers...
  66. ncbi The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease
    J Stakiw
    Department of Medicine, Queen s University, Kingston, ON, Canada
    J Thromb Haemost 6:90-6. 2008
    The effect of exercise on von Willebrand factor (VWF) and ADAMTS-13 levels in individuals with von Willebrand disease (VWD) has never been reported.
  67. doi Polymorphisms in von Willebrand factor gene promoter influence the glucocorticoid-induced increase in von Willebrand factor: the lesson learned from Cushing syndrome
    Alessandra Casonato
    Department of Medical and Surgical Sciences, Second Chair of Internal Medicine, University of Padua Medical School, Padua, Italy
    Br J Haematol 140:230-5. 2008
    ..high-glucocorticoid secretion and an associated hypercoagulable state often involving an increase in von Willebrand factor (VWF)...
  68. pmc Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13
    Wenjing Cao
    Departments of Pathology and Laboratory Medicine, Children s Hospital of Philadelphia, University of Pennsylvania, Philadelphia, PA 19104, USA
    Proc Natl Acad Sci U S A 105:7416-21. 2008
    Proteolytic processing of von Willebrand factor (VWF) by ADAMTS13 metalloproteinase is crucial for normal hemostasis...
  69. doi Sex differences in environmental and genetic factors for hypertension
    Yin Ruixing
    Department of Cardiology, Institute of Cardiovascular Diseases, First Affiliated Hospital, Guangxi Medical University, Nanning, Guangxi, P R China
    Am J Med 121:811-9. 2008
    ..The present study was undertaken to compare the differences in several environmental and genetic factors between men and women in the Hei Yi Zhuang, an isolated subgroup of the Zhuang minority in China...
  70. pmc Platelet glycoprotein Ibalpha forms catch bonds with human WT vWF but not with type 2B von Willebrand disease vWF
    Tadayuki Yago
    Cardiovascular Biology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma 73104, USA
    J Clin Invest 118:3195-207. 2008
    ..Mutations in the vWF A1 domain that cause type 2B von Willebrand disease (vWD) reduce the flow requirement for adhesion...
  71. pmc Von Willebrand factor, type 2 diabetes mellitus, and risk of cardiovascular disease: the framingham offspring study
    David S Frankel
    Division of Cardiovascular Medicine, University of Pennsylvania, Philadelphia, PA, USA
    Circulation 118:2533-9. 2008
    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.
  72. pmc Autosomal dominant C1149R von Willebrand disease: phenotypic findings and their implications
    Almudena Pérez-Rodríguez
    Servicio de Hematologia y Hemoterapia, Complexo Hospitalario, Universitario Juan Canalejo Edificio Hospital Materno Infantil, Carretera del Pasaje s n, La Coruna, Spain
    Haematologica 94:679-86. 2009
    Mutation C1149R in the von Willebrand factor (VWF) gene has been thought to cause autosomal dominant severe type 1 von Willebrand disease (VWD).
  73. pmc Mechanoenzymatic cleavage of the ultralarge vascular protein von Willebrand factor
    Xiaohui Zhang
    Immune Disease Institute, Harvard Medical School, Boston, MA 02115, USA
    Science 324:1330-4. 2009
    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...
  74. doi 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
    Viviana Daidone
    Department of Cardiological, Thoracic and Vascular Sciences, Second Chair of Internal Medicine, University of Padua Medical School, Padua, Italy
    Thromb Res 125:e275-80. 2010
    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 pattern.
  75. pmc Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor
    Veronica H Flood
    Department of Pediatrics, Division of Hematology Oncology, Medical College of Wisconsin, Milwaukee, WI 53201, USA
    Blood 116:280-6. 2010
    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)...
  76. doi Homozygous type 2N R854W von Willebrand factor is poorly secreted and causes a severe von Willebrand disease phenotype
    G Castaman
    Department of Cellular Therapy and Haematology, San Bortolo Hospital, Vicenza, Italy
    J Thromb Haemost 8:2011-6. 2010
    ..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...
  77. pmc Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress
    Christopher G Skipwith
    Biochemistry and Molecular Biophysics Graduate Program, Children s Hospital of Philadelphia and the University of Pennsylvania Medical Center, Philadelphia, Pennsylvania 19104, USA
    J Biol Chem 285:28596-603. 2010
    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...
  78. pmc von Willebrand factor self-association on platelet GpIbalpha under hydrodynamic shear: effect on shear-induced platelet activation
    Kannayakanahalli M Dayananda
    Chemical and Biological Engineering, State University of New York, Buffalo, NY 14260, USA
    Blood 116:3990-8. 2010
    The function of the mechanosensitive, multimeric blood protein von Willebrand factor (VWF) is dependent on its size...
  79. pmc A mechanically stabilized receptor-ligand flex-bond important in the vasculature
    Jongseong Kim
    Immune Disease Institute, Children s Hospital Boston and Department of Pathology, Harvard Medical School, 3 Blackfan Circle, Boston, Massachusetts 02115, USA
    Nature 466:992-5. 2010
    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...
  80. pmc The relationship between levels of von Willebrand factor and mean platelet volume in subjects with isolated impaired fasting glucose
    Feyzi Bostan
    Department of Internal Medicine, Akdeniz University Faculty of Medicine, Antalya, Turkey
    Med Sci Monit 17:PR1-4. 2011
    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).
  81. pmc VWF mutations and new sequence variations identified in healthy controls are more frequent in the African-American population
    Daniel B Bellissimo
    Blood Research Institute, BloodCenter of Wisconsin, Milwaukee, WI, USA
    Blood 119:2135-40. 2012
    Diagnosis and classification of VWD is aided by molecular analysis of the VWF gene...
  82. pmc N-terminal flanking region of A1 domain in von Willebrand factor stabilizes structure of A1A2A3 complex and modulates platelet activation under shear stress
    Matthew Auton
    Section of Cardiovascular Research, Department of Medicine, Baylor College of Medicine, Houston, Texas 77030, USA
    J Biol Chem 287:14579-85. 2012
    b>von Willebrand factor (vWF) mediates platelet adhesion and thrombus formation via its interaction with the platelet receptor glycoprotein (GP)Ibα...
  83. pmc von Willebrand factor (VWF) propeptide binding to VWF D'D3 domain attenuates platelet activation and adhesion
    Sri R Madabhushi
    Departments of Chemical and Biological Engineering
    Blood 119:4769-78. 2012
    Noncovalent association between the von Willebrand factor (VWF) propeptide (VWFpp) and mature VWF aids N-terminal multimerization and protein compartmentalization in storage granules...
  84. doi O-linked glycosylation of von Willebrand factor modulates the interaction with platelet receptor glycoprotein Ib under static and shear stress conditions
    Agata A Nowak
    Department of Haematology, Faculty of Medicine, Hammersmith Hospital Campus, Imperial College London, London, UK
    Blood 120:214-22. 2012
    ..These data demonstrate an important functional role of VWF OLGs under shear stress conditions...
  85. doi von Willebrand factor as new noninvasive predictor of portal hypertension, decompensation and mortality in patients with liver cirrhosis
    Monika Ferlitsch
    Department of Internal Medicine III, Division of Gastroenterology and Hepatology, Medical University of Vienna, Vienna, Austria
    Hepatology 56:1439-47. 2012
    b>von Willebrand factor antigen (vWF-Ag) is elevated in patients with liver cirrhosis, but the clinical significance is unclear...
  86. pmc Association between variations in coagulation system genes and carotid plaque
    David Della-Morte
    Department of Neurology, Miller School of Medicine, University of Miami, Miami, FL, USA
    J Neurol Sci 323:93-8. 2012
    ..The objective of this study is to examine the association between single nucleotide polymorphisms (SNPs) in coagulation system genes and subclinical carotid plaque phenotypes...
  87. pmc Common and rare von Willebrand factor (VWF) coding variants, VWF levels, and factor VIII levels in African Americans: the NHLBI Exome Sequencing Project
    JILL M JOHNSEN
    Department of Medicine, University of Washington, Seattle, WA, USA
    Blood 122:590-7. 2013
    ..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...
  88. pmc The linker between the D3 and A1 domains of vWF suppresses A1-GPIbα catch bonds by site-specific binding to the A1 domain
    Alexander Tischer
    Division of Hematology, Departments of Internal Medicine and Biochemistry and Molecular Biology, Mayo Clinic, Rochester, Minnesota, USA
    Protein Sci 22:1049-59. 2013
    Platelet attachment to von Willebrand factor (vWF) requires the interaction between the platelet GP1bα and exposed vWF-A1 domains...
  89. pmc The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet glycoprotein Ibα
    Lining Ju
    From the Coulter Department of Biomedical Engineering and
    J Biol Chem 288:32289-301. 2013
    Binding of platelet glycoprotein Ibα (GPIbα) to von Willebrand factor (VWF) initiates platelet adhesion to disrupted vascular surface under arterial blood flow...
  90. pmc Secretion of von Willebrand factor by endothelial cells links sodium to hypercoagulability and thrombosis
    Natalia I Dmitrieva
    Renal Cellular and Molecular Biology Section, National Heart, Lung, and Blood Institute, National Institutes of Health, Bethesda, MD 20892
    Proc Natl Acad Sci U S A 111:6485-90. 2014
    ..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...
  91. pmc Selective and signal-dependent recruitment of membrane proteins to secretory granules formed by heterologously expressed von Willebrand factor
    Anastasia D Blagoveshchenskaya
    Vollum Institute L 474, Oregon Health Sciences University, Portland, USA
    Mol Biol Cell 13:1582-93. 2002
    b>von Willebrand factor (vWF) is a large, multimeric protein secreted by endothelial cells and involved in hemostasis...
  92. ncbi Von Willebrand factor, platelets and endothelial cell interactions
    Z M Ruggeri
    Roon Research Laboratory for Arteriosclerosis and Thrombosis, Division of Experimental Thrombosis and Hemostasis, Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, California, USA
    J Thromb Haemost 1:1335-42. 2003
    The adhesive protein von Willebrand factor (VWF) contributes to platelet function by mediating the initiation and progression of thrombus formation at sites of vascular injury...
  93. ncbi A novel type 2A (Group II) von Willebrand disease mutation (L1503Q) associated with loss of the highest molecular weight von Willebrand factor multimers
    L A O'Brien
    Department of Pathology and Molecular Medicine, Richardson Laboratory, Queen s University, Kingston, Ontario, Canada
    J Thromb Haemost 2:1135-42. 2004
    Type 2A von Willebrand disease (VWD) is characterized by decreased platelet-dependent function of von Willebrand factor (VWF) associated with an absence of high-molecular-weight multimers...
  94. pmc Re-establishment of VWF-dependent Weibel-Palade bodies in VWD endothelial cells
    Sandra L Haberichter
    Department of Pediatrics, Medical College of Wisconsin, Milwaukee 53226, USA
    Blood 105:145-52. 2005
    ..mutation in the Chapel Hill strain of canine type 3 VWD that results in premature termination of von Willebrand factor (VWF) protein synthesis...
  95. ncbi Impaired dimerization of von Willebrand factor subunit due to mutation A2801D in the CK domain results in a recessive type 2A subtype IID von Willebrand disease
    Antoine Hommais
    INSERM U 770, Hopital de Bicetre, Universite Paris Sud, France
    Thromb Haemost 95:776-81. 2006
    The CK domain of von Willebrand factor (VWF) is involved in the dimerization of the protein. We identified the homozygous substitution A2801D of the CK domain in two siblings...
  96. ncbi Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13
    J Rayes
    Inserm U770 and Universite Paris Sud, Faculté de Médecine IFR93, Le Kremlin Bicetre, France
    J Thromb Haemost 5:321-8. 2007
    von Willebrand disease (VWD) type 2 is associated with mutations in von Willebrand factor (VWF) that affect its secretion, multimeric pattern, affinity for platelet receptors and clearance of the protein...
  97. ncbi N-linked glycosylation of VWF modulates its interaction with ADAMTS13
    Thomas A J McKinnon
    Haematology Department, Imperial College of London, Hammersmith Hospital Campus, London, United Kingdom
    Blood 111:3042-9. 2008
    ..These data demonstrate that the N-linked glycans of VWF have a modulatory effect on the interaction with ADAMTS13. At least part of this effect is conformational, but steric hindrance may also be important...
  98. doi Detailed von Willebrand factor multimer analysis in patients with von Willebrand disease in the European study, molecular and clinical markers for the diagnosis and management of type 1 von Willebrand disease (MCMDM-1VWD)
    U Budde
    Coagulation Laboratory, Hamburg, Germany
    J Thromb Haemost 6:762-71. 2008
    ..VWD) is a congenital bleeding disorder characterized by a partial quantitative deficiency of plasma von Willebrand factor (VWF) in the absence of structural and/or functional VWF defects...
  99. pmc A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF
    Sara Zanardelli
    Department of Haematology, Imperial College London, Hammersmith Hospital Campus, London, United Kingdom
    Blood 114:2819-28. 2009
    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...
  100. pmc Rapid activation of endothelial cells enables Plasmodium falciparum adhesion to platelet-decorated von Willebrand factor strings
    Daniel J Bridges
    Liverpool School of Tropical Medicine, Liverpool, United Kingdom
    Blood 115:1472-4. 2010
    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 ..

Research Grants64

  1. Von Willebrand Factor Multimer Assembly and Structure
    J Evan Sadler; Fiscal Year: 2012
    b>Von Willebrand factor (VWF) is a multimeric blood glycoprotein that is required for hemostasis. At sites of vascular injury, VWF binds connective tissue and mediates platelet adhesion...
  2. Veronica H Flood; Fiscal Year: 2014
    ..provided by applicant): This project will explore the mechanism(s) responsible for decreased ristocetin-von Willebrand factor (VWF) interactions due to mutations and polymorphisms in the VWF A1 domain, the region of VWF most ..
  3. David Ginsburg; Fiscal Year: 2016
    DESCRIPTION (provided by applicant): von Willebrand factor (VWF) is a key component of the blood coagulation system. Deficiencies of VWF result in the most common inherited bleeding disorder in humans, von Willebrand disease (VWD)...
  4. Prevention of the Complications of Bleeding Disorders Through HTCS
    Regina Butler; Fiscal Year: 2010
    ..bleeding disorder resulting from a deficiency of coagulation factor VIII or factor IX and von Willebrand Disease (VWD) is an inherited bleeding disorder resulting from decreased or abnormal von Willebrand protein...
  5. TRANSGENIC ANALYSIS OF PLATELET RECEPTOR EXPRESSION
    Jerry Ware; Fiscal Year: 2012
    ..The importance of the platelet glycoprotein (GP) Ib-IX/von Willebrand factor axis in this process is well-established and represents an initiating event for platelet adhesion to a ..
  6. Michael R King; Fiscal Year: 2014
    ..platelet translocation due to rapid rates of bond formation and dissociation with surface-immobilized von Willebrand factor (VWF), and 2) the integrin 1221 that supports firm adhesion of platelets to exposed collagen...
  7. Timothy A Springer; Fiscal Year: 2015
    DESCRIPTION (provided by applicant): Von Willebrand factor (VWF) is a concatameric plasma glycoprotein that has multiple functions in hemostasis...
  8. BIOMECHANICS OF UNFOLDING THE A2 DOMAIN OF VON WILLEBRAND FACTOR
    Jin Yu Shao; Fiscal Year: 2012
    DESCRIPTION (provided by applicant): Von Willebrand Factor (VWF) is a multimeric plasma glycoprotein that is critical for both hemostasis and thrombosis...
  9. Structural bases of ADAMTS-13 and VWF A2 interactions
    Cheng Zhu; Fiscal Year: 2013
    ..this and the parent proposal is to elucidate the mechanical regulation of molecular interactions between von Willebrand factor and ADAMTS- 13, which are key interactions on physiological hemostasis and pathological thrombosis...
  10. CHAR MARIE WITMER; Fiscal Year: 2014
    ..In the plasma, FVIII is bound to von Willebrand factor (VWF). VWF is essential for platelet adhesion at the site of vascular injury and thrombus formation...
  11. Xiaoping Du; Fiscal Year: 2016
    ..At sites of vascular injury, GPIb-IX interacts with the subendothelium-bound von Willebrand factor (VWF)...
  12. Effects of Hibernation on Hemostasis in 13-lined Ground Squirrels
    Scott T Cooper; Fiscal Year: 2012
    ..blood clotting, including 3-fold decreases in Factors VIII (FVIII) and IX (FIX), and 10-fold decreases in von Willebrand factor (vWF), neutrophils, and platelets...
  13. MOLECULAR GENETICS OF HUMAN PLATELET GLYCOPROTEINS
    Jose Lopez; Fiscal Year: 2000
    ..is initiated by the interaction of the glycoprotein (GP) Ib-IX-V complex on the platelet surface with von Willebrand factor (vWf) in the subendothelium...
  14. Enabling Structural Studies of Force Activated Adhesion Complexes
    MARK ALAN BLENNER; Fiscal Year: 2012
    ..Specifically, the applicant will study the interaction between the A1-domain of Von Willebrand Factor (VWF-A1) and platelet glycoprotein GPIb a-domain (GPIba)...
  15. Wendy E Thomas; Fiscal Year: 2015
    ..by the binding of platelets via their receptor Glycoprotein Ib (GPIb) to the plasma protein called von Willebrand Factor (VWF) via the A1 domain...
  16. Regulation of factor VIII Levels and Activity by Members of LDL Receptor Family
    Dudley K Strickland; Fiscal Year: 2013
    ..Factor VIII circulates as a complex with its carrier protein von Willebrand factor (vWf)...
  17. Blocking the immune response to Helper-dependent adenovirus vector for Hemophilia
    Masataka Suzuki; Fiscal Year: 2011
    ..To address the potential adaptive immune response to FVIII therapy, we will co-express von Willebrand Factor (vWF) with FVIII...
  18. Structure and Function of the Factor VIII - von Willebrand Factor Complex
    JOHN S LOLLAR; Fiscal Year: 2010
    ..The regulatory processes addressed in this project include the activation of the fVIII-von Willebrand factor (vWf) complex and the control of intrinsic pathway activator function, including the decay of activated ..
  19. Masataka Suzuki; Fiscal Year: 2015
    ..To address the potential adaptive immune response to FVIII therapy, we will co-express von Willebrand Factor (vWF) with FVIII...
  20. Fluorescence Fluctuation Spectroscopy for von Willebrand Factor Multimer Analysis
    Michael J Levene; Fiscal Year: 2010
    ..SUMMARY We propose the development of fluorescence fluctuation spectroscopy (FFS) for the evaluation of von Willebrand Factor (vWF) multimers as a more accurate and reproducible technique in von Willebrand Disease (vWD) diagnosis, ..
  21. Yuandong Peng; Fiscal Year: 2014
    ..is initiated by the binding of the platelet receptor, glycoprotein (GP) Ib-IX complex, to its ligand, von Willebrand factor (VWF)...
  22. Matthew Auton; Fiscal Year: 2015
    DESCRIPTION (provided by applicant): Von Willebrand factor (VWF), a multimeric polymer glycoprotein secreted from vascular endothelial cells and activated platelets, functions to sequester and adhere platelets to the subendothelium and ..
  23. DEVELOPMENT OF A SYNTHETIC VON WILLEBRAND FACTOR
    Dennis Lynch; Fiscal Year: 1992
    ..most common inherited bleeding disorder) is associated with quantitative and qualitative abnormalities in von Willebrand factor (vWF)...
  24. BLOOD COAGULATION--PLATELET-PLASMA PROTEIN INTERACTION
    ZAVERIO RUGGERI; Fiscal Year: 1990
    von Willebrand disease (vWd) subtypes with different structural abnormalities of von Willebrand factor (vWf) have been identified and a vWf tryptic peptide has been isolated which contains the platelet GPIb binding domain...
  25. MOLECULAR GENETIC STUDY OF VONWILLEBRAND FACTOR
    David Ginsburg; Fiscal Year: 2010
    b>von Willebrand factor (VWF) is a central protein in hemostasis. Abnormalities in VWF result in von Willebrand disease (VWD), the most common inherited bleeding disorder in humans...
  26. Natural Variation in VWF and FVIII
    JILL MARIE JOHNSEN; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Levels of the critical plasma clotting proteins von Willebrand Factor (VWF) and Factor VIII (FVIII) are widely variable in humans...
  27. ANALYSIS OF FACTOR VIII COAGULANT ANTIGEN
    Mark Weinstein; Fiscal Year: 1990
    Abnormalities of factor VIII and/or von Willebrand factor (vWF) structure and concentration occur not only in inherited coagulation disorders such as vWF disease and hemophilia A but in acquired disorders including renal dysfunction, ..
  28. Biophysical Analysis of GPIb-VWF Interaction
    Cheng Zhu; Fiscal Year: 2012
    ..The focus is the interaction of the platelet glycoprotein Ib (GPIb) with plasma protein von Willebrand factor (VWF) that is bound to collagen on subendothelial extracellular matrix...
  29. Novel Chaperone Mechanism for Platelet Disorder
    Zihai Li; Fiscal Year: 2010
    ..is through the interaction between platelet glycoprotein Ib- IX-V complex and the endothelial cell-derived von Willebrand factor (VWF), which is tightly regulated physiologically...
  30. The role of VWF and platelets in BBB alterations associated with hypoxia
    GEORGETTE LEILA SUIDAN; Fiscal Year: 2013
    ..b>von Willebrand Factor (VWF) is a glycoprotein that is only synthesized by endothelial cells and megakaryocytes...
  31. Gianluca Interlandi; Fiscal Year: 2016
    ..how oxidation effects alter the structure and function of two key proteins in the hemostatic process, von Willebrand Factor and fibrin...
  32. GPIb Alpha-VWF-A1 bond kinetics in health and disease
    Thomas G Diacovo; Fiscal Year: 2013
    ..bond in order to understand how the physicochemical properties of this interaction may regulate platelet-von Willebrand Factor (VWF) interactions in health and disease, 2) validate proposed biophysical mechanisms by generating and ..
  33. Zaverio M Ruggeri; Fiscal Year: 2014
    ..receptor complex, specifically as they relate to the interaction with its main ligands, thrombin and von Willebrand factor (VWF)...
  34. BRUCE ALAN SULLENGER; Fiscal Year: 2016
    ..Project 2 will translate the first antidote controllable antiplatelet agent, a RNA aptamer against von Willebrand factor (VWF) and its matched antidote oligonucleotide, into phase 1a and 1b studies...
  35. Ultra-Large von Willebrand Factor and Thrombotic Microangiopathy
    Jing Fei Dong; Fiscal Year: 2012
    ABSTRACT Von Willebrand factor (VWF) synthesized in endothelial cells and megkaryocytes is a multimeric glycoprotein that mediates initial platelet tethering to subendothelium exposed at the sites of vessel injury...
  36. Regulation of von Willebrand Factor Reactivity
    JOSE ARON LOPEZ; Fiscal Year: 2012
    ..The only known function of ADAMTS-13 is to cleave von Willebrand factor (VWF), a multimeric protein that when newly secreted spontaneously binds and activates platelets, ..
  37. HUMAN BLOOD PLATELET GLYCOPROTEIN IB
    Alan Michelson; Fiscal Year: 1991
    Platelet adhesion to damaged blood vessel walls is facilitated by the binding of von Willebrand factor (VWF) to platelet membrane glycoprotein Ib (GPIb)...
  38. Functional analysis of ADAMTS13
    David G Motto; Fiscal Year: 2010
    ..The experiments presented focus on the roles that von Willebrand Factor (VWF) and the newly described metalloprotease ADAMTS13 play in pathogenesis of the disease thrombotic ..
  39. WEIBEL PALADE BODY FORMATION AND ROLE IN HEMOSTASIS
    DENISA WAGNER; Fiscal Year: 2007
    ..We propose to examine the role of von Willebrand factor (VWF) in leukocyte adhesion and recruitment to sites of inflammation...
  40. Mechanical Regulation of Binding and Cleavage of VWF by ADAMTS-13
    Cheng Zhu; Fiscal Year: 2012
    ..to study the structural bases and biophysical mechanisms that regulate the molecular interaction between von Willebrand factor (VWF) and VWF-cleaving metalloprotease ADAMTS-13 (A Disintegrin And Metalloprotease with a ThromboSpondin ..
  41. VON WILLEBRAND FACTOR AND THE PATHOPHYSIOLOGY OF TTP
    Joel Moake; Fiscal Year: 1990
    ..S.A. The project is focused on the role of von Willebrand factor (vWF) multimers in the pathophysiology of relapsing and non-relapsing types of TTP...
  42. Rapid Heart Attack Detection using an AC Electrokinetic Device
    Rajaram Krishnan; Fiscal Year: 2011
    ..Markers and antibody stains for identifying EMPs include CD144, von Willebrand Factor (vWF) and Ulex Europaeus Agluttin (UEA), a lectin stain...
  43. Weibel-Palade Bodies: roles in thrombosis, inflammation and stroke
    Denisa D Wagner; Fiscal Year: 2013
    ..propose to continue our studies on the function of Weibel-Palade bodies (WPBs) and their major component, von Willebrand factor (VWF), in normal physiology, responses to injury and in disease models...
  44. Sex Differences in Vascular Markers of Stroke Risk
    Cheryl D Bushnell; Fiscal Year: 2012
    ..include tissue factor, matrix metalloproteinase-9 (MMP-9), vascular cell adhesion molecule-1 (VCAM-1), von Willebrand Factor (vWF), and thrombin-antithrombin III complex (TAT)...
  45. Jun Li; Fiscal Year: 2015
    ..Additionally, we investigated the genetic determinants of plasma von Willebrand Factor (VWF), a plasma protein with an established effect on thrombosis risk, by genotyping and measuring VWF ..
  46. Proteolysis of ultralarge von Willebrand factor and metabolic syndrome-diabetes
    Jing Fei Dong; Fiscal Year: 2011
    ..event for the disease is endothelial injury that expresses and releases adhesion molecules, among them von Willebrand factor (VWF)...
  47. Reducing CVD Risk in Caregivers: A Brief Behavioral Activation Intervention
    Brent T Mausbach; Fiscal Year: 2012
    ..symptoms have been linked with elevated plasma concentrations of D-dimer, Interleukin-6 (IL-6), and von Willebrand Factor (vWF), all of which are associated with increased risk for cardiovascular disease (CVD)...
  48. ASDAMTS-13 and Inflammation
    TRUNG CHANH NGUYEN; Fiscal Year: 2012
    ..More than 50% of these children have a deficiency in von Willebrand factor (VWF)-cleaving protease (a.k.a...
  49. MARK HOWARD GINSBERG; Fiscal Year: 2016
    ..In Project 3, Dr. Ruggeri will build on advances in the structure of von Willebrand Factor (VWF) and platelet GPIb...
  50. William C Aird; Fiscal Year: 2014
    ..Project 1 ("Spatial and temporal dynamics of von Willebrand factor (vWF) gene expression";William C...
  51. Sriram Neelamegham; Fiscal Year: 2015
    DESCRIPTION (provided by applicant): The human blood protein Von Willebrand Factor (VWF) plays a critical role during thrombotic and hemostatic processes by forming a molecular bridge between extra-cellular matrix proteins exposed on ..
  52. Continuous Flow Ventricular Assist Device-Induced Platelet Dysfunction
    TREVOR ARNOULT SNYDER; Fiscal Year: 2013
    ..Up to 30% of CFVAD recipients experience non-surgical bleeding related to an acquired von Willebrand Factor (VWF) deficiency, thought to be caused by the high shear rates blood experiences as it traverses through ..
  53. VON WILLEBRAND FACTOR, FIBRINOLYSIS, & THE ENDOTHELIUM
    Karen Hamilton; Fiscal Year: 1991
    ....
  54. Specific Inhibition of Ectodomain Shedding of GPIb-alpha
    Renhao Li; Fiscal Year: 2012
    ..As the primary receptor in platelets for von Willebrand factor (vWF), GPIb1 is proteolyzed or shed by ADAM17 at a juxtamembrane site, resulting in the release of its N-..
  55. JOSE ARON LOPEZ; Fiscal Year: 2016
    ..from our laboratory, a) one demonstrating that SCD patients have high concentrations of hyperadhesive von Willebrand factor (VWF) in their plasma, the quantity of which correlates with the rate of hemolysis in the patients, b) ..
  56. HEMOSTATIC EFFECTS OF FIBRINOLYTIC THERAPY
    BARBARA ALEVRIADOU; Fiscal Year: 2000
    ..fibrinogen, von Willebrand factor (vWF)...
  57. X-RAYS INDUCE EXOCYTOSIS OF ENDOTHELIAL P-SELECTIN
    Dennis Hallahan; Fiscal Year: 2002
    ..These proteins included CD62P (P-selectin), CD63, Interleukin-8, and von Willebrand factor (vWF)...
  58. THROMBIN AND VWF IN SHIGA-TOXIN-ASSOCIATED HUS
    Thomas Raife; Fiscal Year: 2001
    ..Many investigations have implicated either von Willebrand factor (vWF)-platelet interactions, or thrombin-mediated coagulation pathways as primary pathological mechanisms...
  59. SUBENDOTHELIAL BINDING OF VON WILLEBRAND FACTOR
    JACOB RAND; Fiscal Year: 1991
    ..This platelet adhesion requires von Willebrand factor (vWf), which we previously demonstrated to be present within SE...
  60. VON WILLEBRAND'S DISEASE AND ATHEROSCLEROSIS
    Valentin Fuster; Fiscal Year: 1990
    Our long-term objective is to investigate the role of von Willebrand factor in the interaction of platelets with the arterial wall and in the development of atherosclerosis...
  61. MECHANISMS ON VON WILLEBRAND FACTOR-INDUCED PLATELET ACT
    Michael Kroll; Fiscal Year: 1993
    ..specific aim of this research plan is to determine the nature of the biochemical responses of platelets to von Willebrand factor (vWF) and, in so doing, elucidate the physiologic and pathophysiologic consequences of the vWF/platelet ..
  62. Adhesive Molecular Interactions in Platelet Function
    KOTTAYIL VARUGHESE; Fiscal Year: 2007
    Binding of the A1 domain of von Willebrand factor (VWF A1) to the platelet glycoprotein (GP) Ib-alpha is a central event in the arrest of bleeding but also in the onset of pathological arterial thrombosis...
  63. DDAVP IN CONGENITAL PLATELET FUNCTION DISORDERS
    A Rao; Fiscal Year: 1990
    ..platelet function, and plasma levels of factor VIII activity, factor VIII-related antigen, factor VIII-von Willebrand factor/ristocetin cofactor activity and tissue plasminogen activator (t-PA) in well-characterized patients with ..