von willebrand factor

Summary

Summary: A high-molecular-weight plasma protein, produced by endothelial cells and megakaryocytes, that is part of the factor VIII/von Willebrand factor complex. The von Willebrand factor has receptors for collagen, platelets, and ristocetin activity as well as the immunologically distinct antigenic determinants. It functions in adhesion of platelets to collagen and hemostatic plug formation. The prolonged bleeding time in von Willebrand's disease is due to the deficiency of this factor.

Top Publications

  1. pmc Functional architecture of Weibel-Palade bodies
    Karine M Valentijn
    Department of Molecular Cell Biology, Section Electron Microscopy, Leiden University Medical Center, Leiden, The Netherlands
    Blood 117:5033-43. 2011
  2. ncbi C-reactive protein and other circulating markers of inflammation in the prediction of coronary heart disease
    John Danesh
    Department of Public Health and Primary Care, Institute of Public Health, University of Cambridge, Cambridge, United Kingdom
    N Engl J Med 350:1387-97. 2004
  3. ncbi Immunohistochemical expression of endothelial markers CD31, CD34, von Willebrand factor, and Fli-1 in normal human tissues
    Marc P Pusztaszeri
    Institute of Pathology, Rue du Bugnon 25 1011, Lausanne, Switzerland
    J Histochem Cytochem 54:385-95. 2006
  4. 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
  5. ncbi Collagen XXVIII, a novel von Willebrand factor A domain-containing protein with many imperfections in the collagenous domain
    Guido Veit
    Center for Biochemistry, Center for Molecular Medicine, and Department of Dermatology, Medical Faculty, University of Cologne, D 50931 Cologne, Germany
    J Biol Chem 281:3494-504. 2006
  6. pmc Calcium modulates force sensing by the von Willebrand factor A2 domain
    Arjen J Jakobi
    Crystal and Structural Chemistry, Bijvoet Center for Biomolecular Research, Department of Chemistry, Faculty of Science, Utrecht University, Padualaan 8, 3584 CH Utrecht, The Netherlands
    Nat Commun 2:385. 2011
  7. ncbi High-pressure freezing provides insights into Weibel-Palade body biogenesis
    Helen L Zenner
    MRC Laboratory of Molecular Cell Biology, Cell Biology Unit, and Department of Biochemistry and Molecular Biology, University College London, Gower Street, London, WC1E 6BT, UK
    J Cell Sci 120:2117-25. 2007
  8. 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
  9. ncbi Factor VIII and von Willebrand factor interaction: biological, clinical and therapeutic importance
    V Terraube
    Haemostasis Research Group, Institute of Molecular Medicine, Trinity College, Dublin, Ireland
    Haemophilia 16:3-13. 2010
  10. pmc Biology and physics of von Willebrand factor concatamers
    T A Springer
    Immune Disease Institute, Children s Hospital Boston, Boston, MA, USA
    J Thromb Haemost 9:130-43. 2011

Detail Information

Publications323 found, 100 shown here

  1. pmc Functional architecture of Weibel-Palade bodies
    Karine M Valentijn
    Department of Molecular Cell Biology, Section Electron Microscopy, Leiden University Medical Center, Leiden, The Netherlands
    Blood 117:5033-43. 2011
    Weibel-Palade bodies (WPBs) are elongated secretory organelles specific to endothelial cells that contain von Willebrand factor (VWF) and a variety of other proteins that contribute to inflammation, angiogenesis, and tissue repair...
  2. ncbi C-reactive protein and other circulating markers of inflammation in the prediction of coronary heart disease
    John Danesh
    Department of Public Health and Primary Care, Institute of Public Health, University of Cambridge, Cambridge, United Kingdom
    N Engl J Med 350:1387-97. 2004
    ..We report data from a large study of C-reactive protein and other circulating inflammatory markers, as well as updated meta-analyses, to evaluate their relevance to the prediction of coronary heart disease...
  3. ncbi Immunohistochemical expression of endothelial markers CD31, CD34, von Willebrand factor, and Fli-1 in normal human tissues
    Marc P Pusztaszeri
    Institute of Pathology, Rue du Bugnon 25 1011, Lausanne, Switzerland
    J Histochem Cytochem 54:385-95. 2006
    ..We investigated by immunohistochemistry the expression of CD31, CD34, von Willebrand factor (vWF), and Fli-1 in EC of the major organs and large vessels...
  4. 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...
  5. ncbi Collagen XXVIII, a novel von Willebrand factor A domain-containing protein with many imperfections in the collagenous domain
    Guido Veit
    Center for Biochemistry, Center for Molecular Medicine, and Department of Dermatology, Medical Faculty, University of Cologne, D 50931 Cologne, Germany
    J Biol Chem 281:3494-504. 2006
    Here we describe a novel collagen belonging to the class of von Willebrand factor A (VWA) domain-containing proteins...
  6. pmc Calcium modulates force sensing by the von Willebrand factor A2 domain
    Arjen J Jakobi
    Crystal and Structural Chemistry, Bijvoet Center for Biomolecular Research, Department of Chemistry, Faculty of Science, Utrecht University, Padualaan 8, 3584 CH Utrecht, The Netherlands
    Nat Commun 2:385. 2011
    b>von Willebrand factor (VWF) multimers mediate primary adhesion and aggregation of platelets...
  7. ncbi High-pressure freezing provides insights into Weibel-Palade body biogenesis
    Helen L Zenner
    MRC Laboratory of Molecular Cell Biology, Cell Biology Unit, and Department of Biochemistry and Molecular Biology, University College London, Gower Street, London, WC1E 6BT, UK
    J Cell Sci 120:2117-25. 2007
    ..Tubulation of their major content protein, von Willebrand factor (VWF), is crucial to WPB function, and so we investigated further the relationship between VWF tubule ..
  8. 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 ..
  9. ncbi Factor VIII and von Willebrand factor interaction: biological, clinical and therapeutic importance
    V Terraube
    Haemostasis Research Group, Institute of Molecular Medicine, Trinity College, Dublin, Ireland
    Haemophilia 16:3-13. 2010
    The interaction of factor VIII (FVIII) with von Willebrand Factor (VWF) is of direct clinical significance in the diagnosis and treatment of patients with haemophilia A and von Willebrand disease (VWD)...
  10. pmc Biology and physics of von Willebrand factor concatamers
    T A Springer
    Immune Disease Institute, Children s Hospital Boston, Boston, MA, USA
    J Thromb Haemost 9:130-43. 2011
    Structural specialisations enable von Willebrand factor (VWF) to assemble during biosynthesis into helical tubules in Weibel-Palade bodies (WPB)...
  11. ncbi A dose ranging phase I/II trial of the von Willebrand factor inhibiting aptamer ARC1779 in patients with congenital thrombotic thrombocytopenic purpura
    Petra Jilma-Stohlawetz
    Department of Clinical Pharmacology, Medical University of Vienna, Vienna, Austria
    Thromb Haemost 106:539-47. 2011
    ..the pharmacokinetics and pharmacodynamics and safety of three different administration modes of the anti-von Willebrand factor (VWF) aptamer ARC1779...
  12. ncbi Dynamics and plasticity of Weibel-Palade bodies in endothelial cells
    Mariska G Rondaij
    Department of Plasma Proteins, AMC, University of Amsterdam, Amsterdam
    Arterioscler Thromb Vasc Biol 26:1002-7. 2006
    ..Originally being defined as an intracellular storage pool for von Willebrand factor (VWF), it has recently been shown that an increasing number of other components, including P-selectin, ..
  13. ncbi A shorter von Willebrand factor survival in O blood group subjects explains how ABO determinants influence plasma von Willebrand factor
    Lisa Gallinaro
    Department of Medical and Surgical Sciences, University of Padua Medical School, Italy
    Blood 111:3540-5. 2008
    ABO blood groups greatly influence circulating von Willebrand factor (VWF) levels, and O group subjects have lower VWF values...
  14. ncbi Evidence that differential packaging of the major platelet granule proteins von Willebrand factor and fibrinogen can support their differential release
    S Sehgal
    Department of Physiology and Biophysics, University of Arkansas for Medical Sciences, Little Rock, AR 72205, USA
    J Thromb Haemost 5:2009-16. 2007
    b>von Willebrand factor (VWF) and fibrinogen are major storage proteins of platelet alpha-granules. VWF is synthesized by the megakaryocyte, the cell from which platelets bud, and fibrinogen is delivered to alpha-granules by endocytosis.
  15. ncbi 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...
  16. ncbi Thrombotic microangiopathies
    Joel L Moake
    Baylor College of Medicine, Houston, USA
    N Engl J Med 347:589-600. 2002
  17. 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...
  18. ncbi von Willebrand factor-cleaving protease in thrombotic thrombocytopenic purpura and the hemolytic-uremic syndrome
    M Furlan
    Central Hematology Laboratory, University Hospital, Bern, Switzerland
    N Engl J Med 339:1578-84. 1998
    ..Unusually large multimers of von Willebrand factor, capable of agglutinating circulating platelets under high shear stress, occur in the two conditions...
  19. ncbi Microalbuminuria, von Willebrand factor and fibrinogen levels as markers of the severity in COPD exacerbation
    Mehmet Polatli
    Department of Pulmonology, Adnan Menderes University Hospital, Aydin, Turkey
    J Thromb Thrombolysis 26:97-102. 2008
    ..the vascular endothelium is a major site in which the systemic effect of the inflammation occurs, should von Willebrand Factor, a clotting factor of endothelium origin, and the plasma level of fibrinogen vary with the severity of the ..
  20. ncbi ABO blood group determines plasma von Willebrand factor levels: a biologic function after all?
    P Vince Jenkins
    National Center for Hereditary Coagulation Disorders and Department of Haematology, Institute of Molecular Medicine, St James s Hospital and Trinity College, Dublin, Ireland
    Transfusion 46:1836-44. 2006
    ..attributable to the fact that ABO blood group influences plasma levels of a coagulation glycoprotein named von Willebrand factor (VWF). VWF levels are 25 percent higher in non-O compared to group O individuals...
  21. pmc Rate, extent and concentration dependence of histamine-evoked Weibel-Palade body exocytosis determined from individual fusion events in human endothelial cells
    M Erent
    Medical Research Councils National Institute for Medical Research, Mill Hill, London NW7 1AA, UK
    J Physiol 583:195-212. 2007
    ..67+/-0.24 s (n=6), and a peak fusion rate of approximately 10 WPB s(-1)...
  22. ncbi Mesenchymal stem cells can be differentiated into endothelial cells in vitro
    Joachim Oswald
    Institute of Polymer Research Dresden and The Max Bergmann Center of Biomaterials Dresden, Dresden, Germany
    Stem Cells 22:377-84. 2004
    ..markers like KDR and FLT-1, and immunofluorescence analysis showed typical expression of the von Willebrand factor. The functional behavior of the differentiated cells was tested with an in vitro angiogenesis test kit ..
  23. ncbi von Willebrand factor, endothelial dysfunction, and cardiovascular disease
    U M Vischer
    Department of Cell Physiology and Metabolism and the Department of Rehabilitation and Geriatrics, Faculty of Medicine, University of Geneva, Geneva, Switzerland
    J Thromb Haemost 4:1186-93. 2006
    b>von Willebrand factor (VWF), a glycoprotein involved in arterial thrombus formation, is released into the circulation by secretion from endothelial cells...
  24. ncbi Novel and recurrent mutations clustered in the von Willebrand factor A domain of MATN3 in multiple epiphyseal dysplasia
    Akihiko Mabuchi
    Laboratory for Bone and Joint Diseases, SNP Research Center, RIKEN, 108 8639 Tokyo, Japan
    Hum Mutat 24:439-40. 2004
    ..seven different MATN3 mutations have been identified; all are located within the beta-sheet regions of the von Willebrand factor type A (vWFA) domain, which is encoded by exon 2...
  25. ncbi Adaptation of Staphylococcus aureus to ruminant and equine hosts involves SaPI-carried variants of von Willebrand factor-binding protein
    David Viana
    Centro de Investigación y Tecnología Animal, Instituto Valenciano de Investigaciones Agrarias CITA IVIA, Apdo 187, 12 400 Segorbe, Castellon, Spain
    Mol Microbiol 77:1583-94. 2010
    ..Here, we report first that this differential coagulation activity is due to animal-specific alleles of the von Willebrand factor-binding protein (vWbp) gene, vwb, and second that these vwb alleles are carried by highly mobile ..
  26. pmc Coagulation markers in healthy human subjects exposed to diesel exhaust
    Chris Carlsten
    Department of Medicine, University of Washington, 4225 Roosevelt Ave NE, University of Washington, Campus Box 354695, Seattle, WA 98195, United States
    Thromb Res 120:849-55. 2007
    ..Diesel exhaust is a major contributor to urban PM, and we conducted a controlled human exposure of diesel exhaust in healthy subjects...
  27. pmc Plasma vascular endothelial growth factor, soluble VEGF receptor FLT-1, and von Willebrand factor in glaucoma
    P L Lip
    Haemostasis, Thrombosis and Vascular Biology Unit, University Department of Medicine, City Hospital, Birmingham, UK
    Br J Ophthalmol 86:1299-302. 2002
    ..indices of vascular permeability (vascular endothelial growth factor, VEGF-also an index of angiogenesis, as well as the soluble receptor for VEGF, sFlt-1) and endothelial damage/dysfunction (von Willebrand factor, vWf) in glaucoma.
  28. ncbi Endothelial microparticles induce formation of platelet aggregates via a von Willebrand factor/ristocetin dependent pathway, rendering them resistant to dissociation
    W Jy
    Wallace H Coulter Platelet Lab, Department of Medicine, University of Miami School of Medicine, Miami, FL 33136, USA
    J Thromb Haemost 3:1301-8. 2005
    ..Our recent observation that EMP carry von Willebrand factor (vWf) led us to investigate their interaction with platelets...
  29. ncbi Proteomic screen identifies IGFBP7 as a novel component of endothelial cell-specific Weibel-Palade bodies
    Dorothee van Breevoort
    Department of Plasma Proteins, Sanquin AMC Landsteiner Laboratory, Amsterdam, The Netherlands
    J Proteome Res 11:2925-36. 2012
    ..The main component of WPBs is von Willebrand factor (VWF), a multimeric glycoprotein crucial for platelet plug formation...
  30. ncbi Clearance mechanisms of von Willebrand factor and factor VIII
    P J Lenting
    Department of Clinical Chemistry and Haematology, Laboratory for Thrombosis and Haemostasis, University Medical Center Utrecht, Heidelberglaan 100, 3584 CX Utrecht, The Netherlands
    J Thromb Haemost 5:1353-60. 2007
  31. pmc von Willebrand factor: an emerging target in stroke therapy
    Simon F De Meyer
    Immune Disease Institute, Children s Hospital Boston, Boston, MA, USA
    Stroke 43:599-606. 2012
    ..b>von Willebrand factor (VWF) has long been known to be a key player in thrombus formation at sites of vascular damage...
  32. ncbi Selective release of molecules from Weibel-Palade bodies during a lingering kiss
    Victor Babich
    Division of Molecular Neuroendocrinology, Medical Research Councils MRC, National Institute for Medical Research, London, UK
    Blood 111:5282-90. 2008
    ..The major WPB core proteins are Von Willebrand factor (VWF) and its propolypeptide (Proregion), constituting more than 95% of the content...
  33. pmc Elevated plasma von Willebrand factor and propeptide levels in Malawian children with malaria
    Happy T Phiri
    Malawi Liverpool Wellcome Trust Clinical Research Programme, College of Medicine, Blantyre, Malawi
    PLoS ONE 6:e25626. 2011
    ..of endothelial activation in Ghanaian children with malaria, indicated by elevated plasma levels of both von Willebrand factor (VWF) and its propeptide...
  34. ncbi 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...
  35. ncbi Idiopathic venous thrombosis is related to systemic inflammatory response and to increased levels of circulating markers of endothelial dysfunction
    M K Jezovnik
    Department of Vascular Disease, University Medical Centre Ljubljana, Slovenia
    Int Angiol 29:226-31. 2010
    ..The aim of the present study was to investigate the inflammatory markers and their relationship to idiopathic venous thrombosis...
  36. ncbi 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...
  37. 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).
  38. pmc Efficient inhibition of collagen-induced platelet activation and adhesion by LAIR-2, a soluble Ig-like receptor family member
    Peter J Lenting
    Institut National de la Santé et de la Recherche Médicale INSERM U770 and Univ Paris Sud, Le Kremlin Bicetre, France
    PLoS ONE 5:e12174. 2010
    ..collagen and alpha2beta1 unaffected, but efficiently prevents binding of collagen to Glycoprotein VI and von Willebrand factor. Thus, LAIR-2/Fc has the capacity to interfere with platelet-collagen interactions mediated by ..
  39. ncbi Vascular supply as a discriminating factor for pig preantral follicle selection
    A Martelli
    Department of Comparative Biomedical Sciences, University of Teramo, Teramo, Italy
    Reproduction 137:45-58. 2009
    ..The follicles with this high activity are probably able to reach class 6 becoming dominant structures where somatic and vascular parameters are constantly on high levels and the VA remains the unique differentiating element...
  40. ncbi Primary structure and expression of matrilin-2, the closest relative of cartilage matrix protein within the von Willebrand factor type A-like module superfamily
    F Deak
    Institute of Biochemistry, Biological Research Center of the Hungarian Academy of Sciences, P O Box 521, Szeged H 6701, Hungary
    J Biol Chem 272:9268-74. 1997
    A mouse cDNA encoding a novel member of the von Willebrand factor type A-like module superfamily was cloned...
  41. ncbi von Willebrand factor and its propeptide: the influence of secretion and clearance on protein levels and the risk of venous thrombosis
    A Y Nossent
    Hematology, Hemostasis and Thrombosis Research Center, Leiden University Medical Center, Leiden, The Netherlands
    J Thromb Haemost 4:2556-62. 2006
    ..FVIII levels are determined mainly by von Willebrand factor (VWF)...
  42. ncbi Functional characteristics of N8, a new recombinant FVIII
    M L S Christiansen
    Biopharmaceutical Research Unit, Novo Nordisk A S, Måløv, Denmark
    Haemophilia 16:878-87. 2010
    ..00 (95% confidence interval 0.97-1.03). N8 bound to von Willebrand factor with Kd values of 0.2 nm when measured by ELISA and by surface plasmon resonance...
  43. ncbi Plasma von Willebrand factor levels are an independent risk factor for adverse events including mortality and major bleeding in anticoagulated atrial fibrillation patients
    Vanessa Roldan
    Hematology and Medical Oncology Unit, Hospital Universitario Morales Meseguer, University of Murcia, Murcia, Spain
    J Am Coll Cardiol 57:2496-504. 2011
    The purpose of this study was to evaluate the prognostic value of plasma von Willebrand factor (vWF) levels and fibrin d-dimer in a large cohort of anticoagulated permanent atrial fibrillation (AF) patients.
  44. pmc Neutrophil granulocyte-dependent proteolysis enhances platelet adhesion to the arterial wall under high-shear flow
    N Wohner
    Department of Medical Biochemistry, Semmelweis University, Budapest, Hungary
    J Thromb Haemost 8:1624-31. 2010
    Under high shear stress platelets adhere preferentially to the adventitia layer of the arterial vessel wall in a von Willebrand factor (VWF)-dependent manner.
  45. ncbi Activation of demineralized bone matrix by genetically engineered human bone morphogenetic protein-2 with a collagen binding domain derived from von Willebrand factor propolypeptide
    Bing Chen
    Key Laboratory of Molecular and Developmental Biology, Institute of Genetics and Developmental Biology, Chinese Academy of Sciences, Beijing 100080, China
    J Biomed Mater Res A 80:428-34. 2007
    ..Our data suggested that the collagen-based BMP-2 targeting bone repair system had greater bone inducing ability than DBM loaded with regular BMP-2...
  46. ncbi von Willebrand factor expression in osteosarcoma metastasis
    Kolja Eppert
    Fred A Litwin Centre for Cancer Genetics, Samuel Lunenfeld Research Institute, Mount Sinai Hospital, Toronto, Ontario, Canada
    Mod Pathol 18:388-97. 2005
    ..from 15 patients (including paired primary and metastatic samples from five patients) indicated that von Willebrand factor (vWF) mRNA expression may increase during tumor progression...
  47. 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...
  48. 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...
  49. ncbi Genetic variants associated with Von Willebrand factor levels in healthy men and women identified using the HumanCVD BeadChip
    Delilah Zabaneh
    University College London Genetics Institute, Department of Genetics, Environment and Evolution, London, UK
    Ann Hum Genet 75:456-67. 2011
    We have used the gene-centric Illumina HumanCVD BeadChip to identify common genetic determinants of Von Willebrand factor (vWF) levels in healthy men and women...
  50. ncbi Hemostatic markers of endothelial dysfunction and risk of incident type 2 diabetes: the Framingham Offspring Study
    James B Meigs
    General Medicine Division, Department of Medicine, Massachusetts General Hospital and Harvard Medical School, Boston, 02114, USA
    Diabetes 55:530-7. 2006
    ..of hemostatic markers of endothelial dysfunction, plasminogen activator inhibitor-1 (PAI-1) antigen, and von Willebrand factor (vWF) antigen predicted incident diabetes independent of other diabetes risk factors...
  51. ncbi Involvement of the mitogen-activated protein kinase c-Jun NH2-terminal kinase 1 in thrombus formation
    Alexandre Kauskot
    Centre de Recherche Cardiovasculaire Inserm Lariboisiere, U689 E4, Hopital Lariboisiere, 8 rue Guy Patin, 75010 Paris, France
    J Biol Chem 282:31990-9. 2007
    ..Importantly, at 1500 s(-1), JNK1 acts on thrombus formation on a collagen matrix dependent on GPIb-von Willebrand factor (vWF) interaction but not ADP receptor activation...
  52. 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...
  53. ncbi Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure
    Ralf A Claus
    Dept of Anaesthesiology and Intensive Care Medicine, Friedrich Schiller University, Erlanger Allee 101, D 07747 Jena, Germany
    Thromb Haemost 101:239-47. 2009
    b>Von Willebrand factor (VWF) and related parameters as well as the protease activity regulating its biological activity were measured in plasma of healthy controls and patients with different cause and severity of systemic inflammation to ..
  54. 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...
  55. pmc Crystal structures of the noncatalytic domains of ADAMTS13 reveal multiple discontinuous exosites for von Willebrand factor
    Masashi Akiyama
    aNational Cardiovascular Center Research Institute, Suita, Osaka 565 8565, Japan
    Proc Natl Acad Sci U S A 106:19274-9. 2009
    ADAMTS13 specifically cleaves plasma von Willebrand factor (VWF) and thereby controls VWF-mediated platelet thrombus formation. Severe deficiencies in ADAMTS13 can cause life-threatening thrombotic thrombocytopenic purpura...
  56. pmc Activation-independent platelet adhesion and aggregation under elevated shear stress
    Zaverio M Ruggeri
    The Scripps Research Institute MEM 175, 10550 North Torrey Pines Road, La Jolla, CA 92037, USA
    Blood 108:1903-10. 2006
    ..Platelets interacting with immobilized von Willebrand factor (VWF) aggregate independently of activation when soluble VWF is present and the shear rate exceeds 10 000 ..
  57. ncbi Expression of the endothelial markers PECAM-1, vWf, and CD34 in vivo and in vitro
    Annette M Müller
    Institute of Pathology, University Clinic Bergmannsheil, Ruhr University Bochum, Burkle de la Camp Platz 1, Bochum, 44789 Germany
    Exp Mol Pathol 72:221-9. 2002
    ..Moreover, the need for endothelial markers specific for single microvascular type ECs becomes obvious in order to establish cultures consisting of only one microvascular ECs subpopulation...
  58. ncbi Significance of von Willebrand factor in septic and nonseptic patients with acute lung injury
    Lorraine B Ware
    Division of Allergy, Pulmonary and Critical Care Medicine, T1217 MCN, Vanderbilt University School of Medicine, Nashville, TN 37232 2650, USA
    Am J Respir Crit Care Med 170:766-72. 2004
    ..We hypothesized that plasma levels of von Willebrand factor (VWF), a marker of endothelial activation and injury, would be associated with clinical outcomes in acute ..
  59. ncbi Update on the pathophysiology and classification of von Willebrand disease: a report of the Subcommittee on von Willebrand Factor
    J E Sadler
    Howard Hughes Medical Institute, Washington University, St Louis, MO 63110, USA
    J Thromb Haemost 4:2103-14. 2006
    ..VWD) is a bleeding disorder caused by inherited defects in the concentration, structure, or function of von Willebrand factor (VWF). VWD is classified into three primary categories...
  60. ncbi Mvwf, a dominant modifier of murine von Willebrand factor, results from altered lineage-specific expression of a glycosyltransferase
    K L Mohlke
    Howard Hughes Medical Institute, The University of Michigan, Ann Arbor 48109 0650, USA
    Cell 96:111-20. 1999
    ..as the gain-of-function mechanism responsible for the action of the Mvwf locus, a major modifier of plasma von Willebrand factor (VWF) level in RIIIS/J mice...
  61. pmc Rab27a and MyRIP regulate the amount and multimeric state of VWF released from endothelial cells
    Thomas D Nightingale
    Medical Research Council Laboratory of Molecular Cell Biology, Cell Biology Unit, Department of Cell and Developmental Biology, University College London, London, United Kingdom corrected
    Blood 113:5010-8. 2009
    ..The major cargo protein of WPBs is von Willebrand factor (VWF)...
  62. pmc Actomyosin II contractility expels von Willebrand factor from Weibel-Palade bodies during exocytosis
    Thomas D Nightingale
    Medical Research Council Laboratory for Molecular Cell Biology, University College London, London WC1E 6BT, England, UK
    J Cell Biol 194:613-29. 2011
    ..fusion of individual secretory granules (Weibel-Palade bodies [WPBs]) and subsequent expulsion of von Willebrand factor (VWF) content...
  63. ncbi Similar immune profile in bipolar disorder and schizophrenia: selective increase in soluble tumor necrosis factor receptor I and von Willebrand factor
    Sigrun Hope
    Department of Psychiatry, Østfold Hospital, Eidsberg, Norway
    Bipolar Disord 11:726-34. 2009
    ....
  64. 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...
  65. 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...
  66. pmc Shiga toxin B subunits induce VWF secretion by human endothelial cells and thrombotic microangiopathy in ADAMTS13-deficient mice
    Jing Huang
    Departments of Medicine, Biochemistry, and Molecular Biophysics, Washington University School of Medicine, St Louis, MO 63110, USA
    Blood 116:3653-9. 2010
    ..Recent studies demonstrate that Stx also induces von Willebrand factor (VWF) secretion by human endothelial cells and causes thrombotic thrombocytopenic purpura, a disease with ..
  67. ncbi Mutations in the region encoding the von Willebrand factor A domain of matrilin-3 are associated with multiple epiphyseal dysplasia
    K L Chapman
    Wellcome Trust Centre for Cell Matrix Research, School of Biological Sciences, University of Manchester, Manchester, UK
    Nat Genet 28:393-6. 2001
    ..We have identified two different missense mutations in the exon encoding the von Willebrand factor A (vWFA) domain of matrilin-3 in two unrelated families with MED (EDM5)...
  68. pmc Shear-induced unfolding triggers adhesion of von Willebrand factor fibers
    S W Schneider
    Department of Dermatology, University of Munster, Von Esmarch Strasse 58, 48149 Munster, Germany
    Proc Natl Acad Sci U S A 104:7899-903. 2007
    b>von Willebrand factor (VWF), a protein present in our circulatory system, is necessary to stop bleeding under high shear-stress conditions as found in small blood vessels...
  69. ncbi The physiological function of von Willebrand's factor depends on its tubular storage in endothelial Weibel-Palade bodies
    Gregoire Michaux
    Department of Biochemistry, University College London, Gower Street, London WC1E 6BT, United Kingdom
    Dev Cell 10:223-32. 2006
    ..Thus, a 100-fold compaction of VWF into tubules determines the unique shape of Weibel-Palade bodies and is critical to this protein's hemostatic function...
  70. ncbi Sulfation of Tyr1680 of human blood coagulation factor VIII is essential for the interaction of factor VIII with von Willebrand factor
    A Leyte
    Department of Blood Coagulation, Central Laboratory of the Netherlands Red Cross Blood Transfusion Service, Amsterdam
    J Biol Chem 266:740-6. 1991
    ..VIII light chain was studied with regard to structural requirements for the formation of a functional von Willebrand factor (vWF)-binding site...
  71. 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...
  72. pmc Distribution and evolution of von Willebrand/integrin A domains: widely dispersed domains with roles in cell adhesion and elsewhere
    Charles A Whittaker
    Howard Hughes Medical Institute, Center for Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts 02139, USA
    Mol Biol Cell 13:3369-87. 2002
    ..g., matrilins, cochlin/vitrin, and von Willebrand factor)...
  73. 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.
  74. ncbi Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy
    Myung Chul Chung
    National Center for Biodefense and Infectious Diseases, College of Sciences, George Mason University, 10900 University Boulevard, Manassas, VA 20110, USA
    J Biol Chem 283:9531-42. 2008
    ..In this study we hypothesized that anthrax infection modulates the activity of von Willebrand factor (VWF) and its endogenous regulator ADAMTS13, which play important roles in hemostasis and thrombosis, ..
  75. pmc Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells
    D D Wagner
    J Cell Biol 95:355-60. 1982
    ..These data suggest that the Weibel-Palade bodies of endothelial cells are storage and/or processing organelles for von Willebrand protein...
  76. 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...
  77. pmc Functional self-association of von Willebrand factor during platelet adhesion under flow
    Brian Savage
    Department of Molecular and Experimental Medicine, The Scripps Research Institute, La Jolla, CA 92037, USA
    Proc Natl Acad Sci U S A 99:425-30. 2002
    We have used recombinant wild-type human von Willebrand factor (VWF) and deletion mutants lacking the A1 and A3 domains, as well as specific function-blocking monoclonal antibodies, to demonstrate a functionally relevant self-association ..
  78. ncbi Laboratory diagnosis of von Willebrand disease
    J Patzke
    Siemens Healthcare Diagnostics Products GmbH, 35041 Marburg, Germany
    Hamostaseologie 30:203-6. 2010
    ..It is probable that these GPIbα-binding assays will detect functional VWF defects as the VWF:RCo assay, but are much more sensitive and precise. Fully automated applications on routine analyzers are expected to be commercialized soon...
  79. pmc Shigatoxin triggers thrombotic thrombocytopenic purpura in genetically susceptible ADAMTS13-deficient mice
    David G Motto
    Department of Pediatrics, University of Michigan, Ann Arbor, Michigan 48109, USA
    J Clin Invest 115:2752-61. 2005
    ..These data suggest that microbe-derived toxins (or possibly other sources of endothelial injury), together with additional genetic susceptibility factors, are required to trigger TTP in the setting of ADAMTS13 deficiency...
  80. pmc Hemolytic uremic syndrome-associated Shiga toxins promote endothelial-cell secretion and impair ADAMTS13 cleavage of unusually large von Willebrand factor multimers
    Leticia H Nolasco
    Hematology Research Section, Department of Medicine, Baylor College of Medicine, Houston, TX, USA
    Blood 106:4199-209. 2005
    ..thrombi, acute renal failure, thrombocytopenia, microvascular hemolytic anemia, and plasma levels of von Willebrand factor (VWF)-cleaving protease (ADAMTS13) activity that are within a broad normal range...
  81. ncbi Ristocetin-induced self-aggregation of von Willebrand factor
    Massimiliano Papi
    Istituto di Fisica, Universita Cattolica S Cuore, Rome, Italy
    Eur Biophys J 39:1597-603. 2010
    b>Von Willebrand factor (VWF) is a large multimeric adhesive glycoprotein, with complex roles in thrombosis and hemostasis, present in circulating blood and in secretory granules of endothelial cells and platelets...
  82. ncbi von Willebrand factor promotes leukocyte extravasation
    Björn Petri
    Max Planck Institute for Molecular Biomedicine, Munster, Germany
    Blood 116:4712-9. 2010
    b>von Willebrand factor (VWF) is an important player in hemostasis but has also been suggested to promote inflammatory processes...
  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. ncbi Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor
    A Katsumi
    Howard Hughes Medical Institute, Department of Medicine and Department of Biochemistry and Molecular Biophysics, Washington University School of Medicine, St Louis, MO 63110, USA
    J Biol Chem 275:25585-94. 2000
    b>von Willebrand factor (VWF) is a multimeric glycoprotein that is required for normal hemostasis...
  85. ncbi NAADP links histamine H1 receptors to secretion of von Willebrand factor in human endothelial cells
    Bianca Esposito
    Department of Anatomy, Sapienza University of Rome, Rome, Italy
    Blood 117:4968-77. 2011
    ..Interestingly, H1R-mediated von Willebrand factor (VWF) secretion was completely inhibited by treatment with Ned-19 and by siRNA knockdown of 2-pore channel ..
  86. ncbi Antithrombotic drug candidate ALX-0081 shows superior preclinical efficacy and safety compared with currently marketed antiplatelet drugs
    Hans Ulrichts
    Pharmacology Department, Ablynx NV, Zwijnaarde, Belgium
    Blood 118:757-65. 2011
    ..In conclusion, these preclinical data demonstrate that ALX-0081 combines a high efficacy with an improved safety profile compared with currently marketed antithrombotics. ALX-0081 has entered clinical development...
  87. pmc Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor
    James T B Crawley
    Centre for Haematology, Imperial College London, London, UK
    Blood 118:3212-21. 2011
    b>von Willebrand factor (VWF) is a large adhesive glycoprotein with established functions in hemostasis. It serves as a carrier for factor VIII and acts as a vascular damage sensor by attracting platelets to sites of vessel injury...
  88. ncbi Cytotoxicity of silica nanoparticles through exocytosis of von Willebrand factor and necrotic cell death in primary human endothelial cells
    Alexander T Bauer
    Department of Dermatology, Medical Faculty Mannheim, Heidelberg University, Mannheim 68167, Germany
    Biomaterials 32:8385-93. 2011
    ..310 nm led to significant increase of Weibel-Palade body (WPB) exocytosis, associated with the release of von Willebrand factor (VWF) and the formation of ultralarge fibers (ULVWF)...
  89. pmc Safety and prolonged activity of recombinant factor VIII Fc fusion protein in hemophilia A patients
    Jerry S Powell
    University of California, Davis, Sacramento, CA, USA
    Blood 119:3031-7. 2012
    ..Thus, rFVIIIFc may offer a viable therapeutic approach to achieve prolonged hemostatic protection and less frequent dosing in patients with hemophilia A. This trial was registered at www.clinicaltrials.gov as NCT01027377...
  90. ncbi Regulation of von Willebrand factor-platelet interactions
    Peter J Lenting
    INSERM U 770, 80 rue du General Leclerc, 94276 Le Kremlin Bicetre, France
    Thromb Haemost 104:449-55. 2010
    The formation of thrombi is a multistep process involving several components, including von Willebrand factor (VWF)...
  91. ncbi von Willebrand factor independently predicts long-term survival in patients with pulmonary arterial hypertension
    Steven M Kawut
    Department of Medicine, College of Physicians and Surgeons, Joseph L Mailma School of Public Health, Columbia University, New York, NY, USA
    Chest 128:2355-62. 2005
    We hypothesized that higher plasma levels of von Willebrand factor (vWF) and reduced ristocetin cofactor activity (RCA)/vWF ratios at baseline and follow-up would be associated with an increased risk of death in patients with pulmonary ..
  92. pmc Force-induced cleavage of single VWFA1A2A3 tridomains by ADAMTS-13
    Tao Wu
    Woodruff School of Mechanical Engineering, Georgia Institute of Technology, Atlanta, GA 30332, USA
    Blood 115:370-8. 2010
    ..a thrombospondin type 1 motifs 13 (ADAMTS-13) regulates hemostasis by cleaving the folded A2 domain of von Willebrand factor (VWF). The cleavage is regulated by forces as it occurs in flowing blood...
  93. ncbi Laboratory tests for measurement of von Willebrand factor show poor agreement among different centers: results from the United Kingdom National External Quality Assessment Scheme for Blood Coagulation
    Steve Kitchen
    United Kingdom National External Quality Assessment Scheme NEQAS for Blood Coagulation, Sheffield, United Kingdom
    Semin Thromb Hemost 32:492-8. 2006
    In recognition of the importance of von Willebrand factor (vWF) testing in the diagnosis of von Willebrand disease (vWD), the United Kingdom National External Quality Assessment Scheme for Blood Coagulation regularly distributes samples ..
  94. ncbi Associations of von Willebrand factor, fibrin D-dimer and tissue plasminogen activator with incident coronary heart disease: British Women's Heart and Health cohort study
    Margaret May
    Department of Social Medicine, University of Bristol, Canynge Hall, Whiteladies Road, Bristol, UK
    Eur J Cardiovasc Prev Rehabil 14:638-45. 2007
    Associations of three markers of thrombotic tendency, von Willebrand factor, tissue plasminogen activator antigen and fibrin D-dimer, with coronary heart disease have been reported in meta-analyses...
  95. 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
    ..We conclude that in type 2B vWD, prolonged lifetimes of vWF bonds with GPIbalpha on circulating platelets may allow ADAMTS-13 to deplete large vWF multimers, causing bleeding...
  96. ncbi Plasma fibronectin depletion enhances platelet aggregation and thrombus formation in mice lacking fibrinogen and von Willebrand factor
    Adili Reheman
    Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada
    Blood 113:1809-17. 2009
    ..showed that platelet aggregation and thrombus formation occurred in mice lacking both fibrinogen (Fg) and von Willebrand factor (VWF) and that plasma fibronectin (pFn) promoted thrombus growth and stability in injured arterioles in ..
  97. ncbi ADAMTS13 deficiency with elevated levels of ultra-large and active von Willebrand factor in P. falciparum and P. vivax malaria
    Quirijn de Mast
    Department of General Internal Medicine, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands
    Am J Trop Med Hyg 80:492-8. 2009
    A deficiency in ADAMTS13 (a von Willebrand factor [VWF] cleaving protease) is associated with accumulation of prothrombogenic unusually large VWF multimers (UL-VWF) in plasma...
  98. 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...
  99. ncbi von Willebrand factor activation, granzyme-B and thrombocytopenia in meningococcal disease
    M J Hollestelle
    Department of Clinical Chemistry and Haematology, University Medical Centre Utrecht, Utrecht, The Netherlands
    J Thromb Haemost 8:1098-106. 2010
    ..During invasive meningococcal disease, severe thrombocytopenia is strongly associated with a poor outcome...
  100. 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.
  101. ncbi High dose dexamethasone increases circulating P-selectin and von Willebrand factor levels in healthy men
    Bernd Jilma
    Department of Clinical Pharmacology TARGET, Medical University Vienna, Austria
    Thromb Haemost 94:797-801. 2005
    ..Hence,we measured changes of a specific endothelial cell marker (von Willebrand factor [vWF]) and of a platelet marker (soluble P-selectin) by infusing therapeutic doses of dexamethasone (0...

Research Grants72

  1. Autonomic, Endothelial, and Inflammatory Correlates of Sleep Duration
    MERCEDES RENEE CARNETHON; Fiscal Year: 2012
    ..examination to have resting parasympathetic function measured, and biomarkers of endothelial function (von Willebrand factor and E-selectin) and adipocytokines that represent insulin resistance (adiponectin, leptin, resistin, tumor ..
  2. Comprehensive biomarker study to capitalize on existing GWAS in 10K South Asians
    Danish Saleheen; Fiscal Year: 2010
    ..IL-6, sTNFRII, sVCAM-1, sICAM-1, serum amyloid A, MMP-9, E-selectin, P-selectin, CD40 ligand, fibrinogen, von Willebrand factor, and D-dimer)...
  3. A Clinical Trial of Aspirin and Simvastatin in Pulmonary Arterial Hypertension
    Steven Kawut; Fiscal Year: 2009
    ..We hypothesize that simvastatin will increase brachial artery flow-mediated dilatation and lower von Willebrand factor compared to placebo. 4) To determine whether aspirin affects platelet function in patients with PAH...
  4. Cyr61/CCN1-Induced Angiogenesis and Vasculogenesis in the Retina
    Brahim Chaqour; Fiscal Year: 2010
    ..consisting of 4 distinct domains: an insulin-like growth factor binding protein (IGFBPs) domain, the von Willebrand factor type C (vWFC) repeat, the thrombospondin type I (TSP1) repeat and a C-terminal (CT) cystine-knot motif...
  5. 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)...
  6. 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...
  7. Prevention and therapy of Staphylococcus aureus infections
    Olaf Schneewind; Fiscal Year: 2010
    ..These technologies will be developed with the goal of generating a vaccine and immune therapeutics, thereby providing protection against S. aureus infections and improving global health. ..
  8. 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 ..
  9. 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...
  10. Endothelial Dysfunction and the RV in the Multi-Ethnic Study of Atherosclerosis
    Steven M Kawut; Fiscal Year: 2010
    ..ejection fraction in MESA by gender, race, and ethnicity, 2) to determine whether plasma measures, such as von Willebrand factor, soluble thrombomodulin, C-reactive protein, interleukin-6, and fibrinogen, are associated with RV mass ..
  11. Reasons for Racial Disparities in Venous Thromboembolism
    NEIL ADRIAN ZAKAI; Fiscal Year: 2010
    ..to a follow- up proposal to obtain funding to evaluate novel biomarkers of VTE risk such as factor VIII, von Willebrand factor levels, and genetic factors in African-Americans versus European-Americans...
  12. Mechanism of Staphylocoagulase-activated Blood Clotting
    Paul E Bock; Fiscal Year: 2013
    ..goals are to define the mechanisms of blood clotting by Staphylococcus aureus staphylocoagulase (SC) and von Willebrand factor binding-protein (vWbp), and their roles in acute infective endocarditis (AIE)...
  13. MOLECULAR &CELLULAR MECHANISMS IN TRANSFUSION MEDICINE
    Peter J Newman; Fiscal Year: 2013
    ..In PROJECT 5, Robert Montgomery proposes to examine the ability of von Willebrand factor to direct and stabilize factor Vlll in a series of novel large- and small-animal gene therapy protocols...
  14. Receptor disulfide allosteric regulation of anthrax toxin action
    Jianjun Sun; Fiscal Year: 2013
    ..R2-Ig induced a significant conformational change in the distal PA-binding domain of the receptor, namely von Willebrand Factor A domain (R2-VWA)...
  15. Early Insulin Therapy and Development of ARDS
    Michelle Ng Gong; Fiscal Year: 2011
    ..Day 1 and 3 of the study and plasma levels of free fatty acids, tumor necrosis factor-a, interleukin-6 and Von Willebrand Factor antigen will be determined by liquid and gas chromatography and by ELISA...
  16. 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...
  17. Single molecule measurements on von Willebrand factor A1 and A2 domains
    Timothy A Springer; Fiscal Year: 2013
    DESCRIPTION (provided by applicant): Von Willebrand factor (VWF) is a concatameric plasma glycoprotein that has multiple functions in hemostasis...
  18. Molecular Genetic Study of von Willebrand Factor
    David Ginsburg; Fiscal Year: 2013
    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)...
  19. 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...
  20. Multi-stage multi-antigen Vaccine for interrupting Malaria Transmission
    B Kim Lee Sim; Fiscal Year: 2013
    ..To further enhance VIMT effects, we will assess Pf von Willebrand factor A domain-related protein (PfWARP), a highly conserved, soluble ookinete specific protein that we have ..
  21. Weibel - Palade Bodies - Sentinels of Acute Ischemia
    Michael S Goligorsky; Fiscal Year: 2013
    ..of Weibel-Palade bodies and release of their constituents, such as angiopoietin-2, interleukin- 8 and von Willebrand factor resulting 3) in the systemic mobilization of EPC and engraftment of the affected organ improving its ..
  22. Model of Platelet Adhesion and Thrombus Formation
    Michael R King; Fiscal Year: 2013
    ..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...
  23. Signaling Mechanism of Platelet Glycoprotein Ib-IX
    Xiaoping Du; Fiscal Year: 2013
    ..At sites of vascular injury, GPIb-IX interacts with the subendothelium-bound von Willebrand factor (VWF)...
  24. 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)...
  25. Identifying novel genetic risk factors for venous thromboembolism (VTE)
    Jun Li; Fiscal Year: 2013
    ..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 ..
  26. 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, ..
  27. Regulation of Platelet Glycoprotein (GP) Ib-IX Complex Function by Membrane Lipid
    Yuandong Peng; Fiscal Year: 2013
    ..is initiated by the binding of the platelet receptor, glycoprotein (GP) Ib-IX complex, to its ligand, von Willebrand factor (VWF)...
  28. Structure and Stability Studies of Factor VIII to Overcome the Immune Response
    PAUL CLINTON SPIEGEL; Fiscal Year: 2010
    ..The C2 domain of factor VIII is essential for proper interactions with von Willebrand factor and the membrane surface of activated platelets, and is a major epitope that is recognized by inhibitory ..
  29. 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)...
  30. Novel Regulators of Endothelial Exocytosis
    Charles J Lowenstein; Fiscal Year: 2013
    ..In contrast, endothelial cells secrete von Willebrand Factor into the media, a protein easy to measure in a high-throughput format...
  31. 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)...
  32. Structure and function of platelet membrane glycoproteins
    Zaverio M Ruggeri; Fiscal Year: 2013
    ..receptor complex, specifically as they relate to the interaction with its main ligands, thrombin and von Willebrand factor (VWF)...
  33. 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...
  34. 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...
  35. 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...
  36. EPIDEMIOLOGY OF VENOUS THROMBOSIS AND PULMONARY EMBOLISM
    Aaron R Folsom; Fiscal Year: 2013
    ..whites to identify low frequency variants associated with important plasma intermediate phenotypes (aPTT, von Willebrand factor, FVIII, FXI, and Y fibrinogen), and to evaluate any significant variants for associations wih VTE...
  37. 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...
  38. Blocking the immune response to HDAd for Hemophilia A gene therapy
    Masataka Suzuki; Fiscal Year: 2013
    ..To address the potential adaptive immune response to FVIII therapy, we will co-express von Willebrand Factor (vWF) with FVIII...
  39. Mechanisms of Immunogenicity of Factor VIII
    Shannon L Meeks; Fiscal Year: 2013
    ..a site of inflammation where a clot is forming and where fVIII is released from its large carrier protein von Willebrand factor, will be characterized by comparing the immunogenicity of fVIII with an inactive form of fVIII in a murine ..
  40. Cell Adhesion Mechanisms in Vascular Disease &Thrombosis
    MARK HOWARD GINSBERG; Fiscal Year: 2013
    ..In Project 3, Dr. Ruggeri will build on advances in the structure of von Willebrand Factor (VWF) and platelet GPIb...
  41. Endothelial Cell Phenotypes in Health and Disease
    William C Aird; Fiscal Year: 2013
    ..Project 1 ("Spatial and temporal dynamics of von Willebrand factor (vWF) gene expression";William C...
  42. 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, ..
  43. 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...
  44. Chemistry and Biology of Coagulation
    Sriram Krishnaswamy; Fiscal Year: 2013
    ..Project 3 (Zheng) investigates the link between coagulation and von Willebrand Factor processing by investigating the regulation of ADAMTS13 function by factor VIII and shear stress...
  45. VON WILLEBRAND FACTOR STRUCTURE AND FUNCTION UNDER FLUID FLOW
    Sriram Neelamegham; Fiscal Year: 2013
    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 ..
  46. Role of Vwc2-assisted activin signaling in bone formation
    Yoshiyuki Mochida; Fiscal Year: 2010
    ..By a bioinformatics approach, we have identified a novel member of CKP family, "Vwc2 (von Willebrand factor C domain containing 2)", and explored its potential functions...
  47. Structural analysis of oxidation effects on the hemostatic process
    Gianluca Interlandi; Fiscal Year: 2013
    ..how oxidation effects alter the structure and function of two key proteins in the hemostatic process, von Willebrand Factor and fibrin...
  48. VWF Multimer Distribution a Phenotypic Modifier of Hemophilia A
    CHAR MARIE WITMER; Fiscal Year: 2013
    ..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...
  49. Thrombus Formation In Vivo
    Bruce Furie; Fiscal Year: 2012
    ..Barbara Furie will study the combined roles of GPVI/PAR4 and PAR4/von Willebrand factor in platelet activation during thrombus formation, the contribution of cellular sources of von Willebrand ..
  50. Functional Implications of VWF A1 Domain Polymorphisms
    Veronica H Flood; Fiscal Year: 2013
    ..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 ..
  51. Passivating Proteins in Implantable Glucose Sensors
    W Ward; Fiscal Year: 2004
    ..The proteins will include von Willebrand factor, high molecular weight kininogen, albumin, and hemoglobin...
  52. 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 ..
  53. 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...
  54. Prevention of the Complications of Bleeding Disorders Through HTCS
    Regina Butler; Fiscal Year: 2010
    ....
  55. Structural Vaccinology of the Malaria Sporozoite Surface Sheath
    Timothy A Springer; Fiscal Year: 2013
    ..2. We will solve the crystal structure of the tandem von Willebrand factor A (VWA) and TSR domains in TRAP...
  56. Genetic Epidemiological Study of Venous Thromboembolism and Hemostatic Factors
    Weihong Tang; Fiscal Year: 2012
    ..The intermediate phenotypes include plasma levels of factor VIIIc, von Willebrand factor, and activated partial thromboplastin time...
  57. Structure and Function of Platelet Glycoprotein Ib-IX-V Complex
    Renhao Li; Fiscal Year: 2013
    ..It is identified primarily as the receptor for a number of hemostatically important proteins including von Willebrand factor and thrombin. It has also been implicated in the genesis and clearance of platelets...
  58. 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...
  59. Structural Vaccinology of the Malaria Sporozoite Surface Sheath
    Timothy A Springer; Fiscal Year: 2011
    ..2. We will solve the crystal structure of the tandem von Willebrand factor A (VWA) and TSR domains in TRAP...
  60. 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...
  61. FASEB SRC on Proteases in Hemostasis and Vascular Biology
    JAMES ANDREW HUNTINGTON; Fiscal Year: 2013
    ..structure, function and regulation;2) Platelet adhesion;3) Fibrin(ogen)(olysis);4) Platelet signaling;5) von Willebrand Factor, TTP and proteases;6) Proteases and inhibitors in host defense;7) The O'Mics: Genomics/proteomics/..
  62. Thermodynamics of the Conformational Activation of Von Willebrand Factor
    Matthew Auton; Fiscal Year: 2013
    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 ..
  63. VON WILLEBRAND FACTOR, FIBRINOLYSIS, & THE ENDOTHELIUM
    Karen Hamilton; Fiscal Year: 1991
    ....
  64. HEMOSTATIC EFFECTS OF FIBRINOLYTIC THERAPY
    BARBARA ALEVRIADOU; Fiscal Year: 2000
    ..fibrinogen, von Willebrand factor (vWF)...
  65. 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)...
  66. Mechanistic Role of NELL-1 in Premature Suture Closure
    Kang Ting; Fiscal Year: 2003
    ..is a secretory protein with a signal peptide, an NH2-terminal thrombospondin (TSP)-like module, five von Willebrand factor C domains, and six epidermal growth factor-like domains...
  67. Structural studies of blood coagulation proteins
    Barry Stoddard; Fiscal Year: 2006
    ..Prior to its own activation, factor VIII circulates in a tight, stable complex with von Willebrand Factor. A wide variety of inheritable coagulation deficiencies are associated with mutations in factor VIII (..
  68. INTEGRATIVE APPROACH TO CORONARY THROMBOSIS
    Robert Montgomery; Fiscal Year: 2001
    ..is that: following a plaque rupture or damage to the endothelium, the circulating multimeric protein von Willebrand factor (vWF) adheres to exposed subendothelium; this is followed by platelet adherence mediated via the GPIba ..
  69. 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...
  70. 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...
  71. 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)...
  72. 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 ..