Property Summary

NCBI Gene PubMed Count 747
PubMed Score 8726.33
PubTator Score 5448.68

Knowledge Summary

Patent

No data available

TINX Plot

  Disease (7)

Disease Target Count Z-score Confidence
Carcinoma 2147 0.0 1.0
Disease Target Count Z-score Confidence
Von Willebrand's disease 19 8.843 4.0

Expression

  Differential Expression (19)

Disease log2 FC p
urothelial carcinoma 1.700 1.4e-03
osteosarcoma -2.556 1.1e-02
ulcerative colitis 2.100 1.7e-05
adrenocortical carcinoma 2.180 1.1e-08
non-small cell lung carcinoma -2.100 5.9e-33
intraductal papillary-mucinous adenoma (... -2.500 3.2e-05
intraductal papillary-mucinous carcinoma... -2.500 2.6e-04
intraductal papillary-mucinous neoplasm ... -2.300 6.0e-04
lung cancer -3.900 3.9e-05
breast carcinoma -1.300 1.7e-03
interstitial cystitis 1.800 2.8e-05
lung adenocarcinoma -3.100 1.9e-12
group 3 medulloblastoma 1.200 2.1e-02
lung carcinoma -1.900 1.0e-09
gastric carcinoma 1.500 1.3e-02
ductal carcinoma in situ -1.600 2.4e-04
invasive ductal carcinoma -2.500 1.1e-04
ovarian cancer 1.500 2.0e-05
Down syndrome 1.100 1.3e-02

 CSPA Cell Line (2)

Protein-protein Interaction (3)

Gene RIF (663)

PMID Text
27060324 rs216293T/G and rs1063857T/C polymorphisms of the vWF gene are associated with the risk for coronary artery disease.
26871104 Study compared the force-induced domain unfolding of recombinant dimeric VWF with recombinant VWF multimers
26827609 Based on prediction scores, four variants, namely, P1266L, H1268D, C1272R, and C1272F, were predicted as highly deleterious from a pool of 72 nsSNPs/variants in A1 domain of VWD belonging to type 2A and 2B
26773038 Glycan stabilization of the VWF A2 domain acts together with the Ca(2+)binding site and vicinal cysteine disulfide bond to control unfolding and ADAMTS13 proteolysis.
26744078 Clonal analysis of cultured endothelial cells reveals that dynamic mosaic heterogeneity in vWF expression is controlled by a low-barrier, noise-sensitive bistable switch that involves random transitions in the DNA methylation status of the VWF promoter.
26677223 These studies reveal a novel rheological strategy in which the incorporation of a single glycine within the GPIbalpha binding interface of normal VWF enhances the probability of local unfolding
26670633 direct binding of PDIA1 to VWF, is reported.
26645283 results strongly argue against apoptosis as a mechanism for the thrombocytopenia of severe VWD-2B exhibiting the VWF/p.V1316M mutation
26616301 An in vitro model for LVAD associated aVWD demonstrated that ADAMTS-13 and platelets contribute to the depletion of HMWM of VWF.
26600159 In the ACS patients, associations between ABO antigens and von Willebrand factor (VWF, P = 9.2 x 10-13), and soluble tissue factor (sTF, P = 8.6 x 10-4) were also found.
26595154 ADAMTS13 is the key protease that regulates the multimeric state of VWF. Without ADAMTS13, VWF multimers can grow to pathologically large sizes. This is a risk factor for the life-threatening condition thrombotic thrombocytopenic purpura (TTP)
26581637 free thiol groups are shown to be involved in VWF binding to both collagen III and platelet GP1b receptor.
26552698 ApoA-I requires lipid to stabilize VWF under shear.
26473492 Stimulated GMVECs and HUVECs were found to secrete cell-anchored ultra-large VWF strings covered with bound FVIII.
26345337 Type 2B mutations localized in the A1 domain could enhance the sensitivity to ADAMTS13-mediated proteolysis. When GPIbalpha participated, there was a dramatically increased proteolytic cleavage of VWF by ADAMTS13 to rVWF-WT, excluding some type 2B mutants.
26307534 This study is the first to demonstrate that extracellular Hb directly affects the GPIbalpha-VWF interaction in thrombosis, and describes another mechanism by which hemolysis is connected to thrombotic events.
26266817 SNAP23 Regulates Endothelial Exocytosis of von Willebrand Factor.
26245874 Large cohort of Spanish von Willebrand disease patients in whom VWF mutations have been identified.
26228839 No association between cognition and gene polymorphisms involved in thrombosis and haemostasis
26223854 vWF assembles into bundles or meshes in microvessels depending on vessel geometry and flow characteristics. vWF bundles and webs bind platelets, WBCs and RBCs, obstructing flow.
26178390 Pathological shear rates generate fibrous VWF from plasma. Its elastic modulus is ~50 MPa. It is non-amyloid, tPA-resistant, plasmin-sensitive, and accumulates procoagulant factors to drive fibrin formation. Platelets roll and adhere to it.
26168189 Increased von Willebrand factor, ADAMTS13, and D-Dimer levels and decreased ADAMTS13 activity/ADAMTS13 levels ratio are associated with renal dysfunction in patients with type 1 diabetes
26121272 Stroke in human immunodeficiency virus infection is associated with a prothrombotic state, characterized by elevated von Willebrand factor and low ADAMTS13 levels
26088471 4 mutations in the D1 domain of VWF impair the multimerization of VWF by downregulating the oxidoreductase of the propeptide, disrupt the transport of VWF from the ER to the Golgi and inhibit the basal and regulated secretion of VWF; these defects contribute to the quantitative loss of VWF, leading to the bleeding diathesis of von Willebrand disease
26047609 a genetic link between EGLN1 and VWF in a constitution specific manner which could modulate thrombosis/bleeding susceptibility and outcomes of hypoxia, is reported.
26019279 The observed variability in VWD phenotypes could in part be determined by the extent of mutant monomer incorporation in the final multimer structure of plasma VWF.
25983111 The VWF nets as well as the platelet-VWF conglomerates are controlled by the cleaving protease ADAMTS13 within minutes under high shear flow.
25973092 the relationship between vWF and claudin-5, which are indicators of endothelial cell dysfunction and tight junction activity, may be a predictor of disease activity in rheumatoid arthritis.
25916953 data suggest that increased levels of VWF and reduced levels of ADAMTS13 activity may contribute to the pathogenesis of cerebral infarction.
25904363 The p.R1819_C1948delinsS mutation has both haemorrhagic features (defective collagen binding, reduced VWF levels) and prothrombotic (ADAMTS13 resistance) features, and the latter probably mitigate the patient's bleeding symptoms.
25886574 Data showed that gastric cancer-related plasma vWF activity levels become substantially elevated in the late stage of disease and may be regulated by the VEGF-VEGFR2 signal pathway contributing probably to gastric cancer progression.
25876231 Report changes in von Willebrand factor levels in patients with essential thrombocytopenia and controlled platelet count.
25846964 Decrease of ADAMTS13 is associated with the risk of platelet thrombosis within the allograft during Lung Transplantation.
25832887 Genetic variations in STXBP5 and CLEC4M are associated with VWF level variation in type 1, but not in type 2 von Willebrand disease.
25810255 Data show that force can switch the kinetics of bond formation between A1 domain of von Willebrand factor (VWF) and glycoprotein Ibalpha (GPIbalpha).
25782102 Unconjugated bilirubin directly inhibits ADAMTS13's ability to cleave both peptidyl and native VWF substrates.
25780857 Report analysis of VWF phenotype/genotype to supplement standard F8 analysis for patients diagnosed with hemophilia A.
25779970 Gene-centric approach identifies new and known loci for FVIII activity and VWF antigen levels in European Americans and African Americans.
25756206 Patients with type 1 VWD experience age-related increases to VWF plasma levels and VWF activity which can result in normalization of VWF levels.
25673639 von Willebrand factor mutation is associated with von Willebrand disease.
25670067 In patients with implantation of a transcatheter aortic valve or of a CF left ventricular assist device, induction/recovery of HMW multimers occurs in minutes. Those with residual high shear or aortic regurgitation did not recover HMW multimers.
25650553 Clearance differences between blood group O and non-blood group O individuals may therefore be related to the blood group status of the individual rather than the ABH antigen loading on VWF itself.
25646961 Identified is a novel single nucleotide polymorphism - clinical parameter interaction between rs7965413 and platelet count that was significantly associated with metabolic syndrome.
25612915 This brief review will highlight knowledge acquired and key problems that remain to be solved to elucidate fully the role of VWF in normal haemostasis and pathological thrombosis. [human]
25608503 CD40 ligand induces von Willebrand factor release from endothelial cells
25605439 Interaction between VWF and FVIII in treating VWD.
25512528 Thus, ADAMTS13 is regulated by substrate-induced allosteric activation, which may optimize VWF cleavage under fluid shear stress in vivo
25498844 ADAMTS13 activity is reduced and VWF:Ag expression is increased within 4 weeks of TIA or ischaemic stroke onset, and can promote enhanced platelet adhesion and aggregation in response to stimulation with collagen and ADP via VWF-mediated pathways
25472874 In acute coronary syndrome and stable angina pectoris patients, high VWF levels are predictive of adverse cardiovascular outcome and death during one-year follow-up.
25439775 Supraphysiologic shear stress alone causes physical demolition of large von Willebrand factor multimers into smaller von Willebrand factor multimers i patients with left ventricular device implantation.
25425019 In individuals with borderline VWF, the two strongest predictors of VWD diagnosis are low VWF:RCo levels (particularly in those with blood group non-O) and female sex.
25420483 PON1 and VWF are detectable markers that may predict the severity of stenosis.
25397410 The effect of reduced platelet count in cirrhotic patients can at least be partly compensated by increased vWF levels.
25395424 Normal plasma VWF multimers prevent complement activation and steer the complement pathway toward generation of inactivated C3b (iC3b).
25390649 von Willebrand factor and IL-6, but not P-selectin in the peripheral and cardiac radiofrequency catheter ablation of atrial fibrillation circulation are affected by
25387000 Von Willebrand factor is elevated in and is associated with a fatal outcome in Sudan virus-infected pediatric patients.
25381443 The C domains of VWF and the E domain of fibrin monomers are involved in the incorporation of VWF during the polymerization of fibrin.
25363217 von Willebrand immunohistochemistry shows widespread heavy damage of capillary endothelial cells in the pathogenesis of sarcoidosis.
25341886 Whereas VWF-D'D3 is the major regulator of soluble VWF binding to platelet GpIbalpha, both the D'D3-domain and N-terminal peptide regulate platelet translocation and thrombus formation.
25305202 collagen and von Willebrand factor-dependent platelet functions are affected by ibrutinib
25300811 We have assessed several VWF antigen and activity assays.
25300810 This ELISA assay might be used as an additional confirmation tool in the diagnostic procedure in patients affected by AVWS or in the follow-up of congenital and acquired patients exposed to replacement therapy.
25274249 Letter: thrombin generation in the presence of platelets is sensitive to the activation status of von Willebrand factor.
25250329 The vWA and TPOX microsatellites are good candidate biomarkers in venous thromboembolism diseases and could help to elucidate their origins.
25230768 Suggest cysteine 584 has a role in the release of VWF and its targeting to pseudo-Weibel Palade bodies in vitro, as well as in its storage and release by endothelial cells in vivo.
25201004 assay may serve as a valuable research tool to investigate the biochemical characteristics of VWF and ADAMTS13 under shear flow.
25196897 Hemophilia A patients on rFVIIIFc prophylaxis undergoing extended-interval dosing maintains low bleeding rates and correlates with von Willebrand factor levels.
25185554 Results suggest that specific secondary structure elements of the A1 domain regulate GPIbalpha binding and the strength of vWF-platelet interactions, which affects the vWD functional phenotype and the severity of thrombocytopenia
25113276 Systemic inflammation complicating advanced cirrhosis is accompanied by reduced activity of ADAMTS13 promoting a prothrombotic function of von Willebrand factor, which can be employed to predict clinical outcome.
25105915 VWF and FVIII were related to circulating blast cells and steroids therapy through lysis of lymphoblasts results in reduction of both factors, with risk of thrombosis during induction with marked disintegration of malignant cells.
25103891 HEK293 cells expressing VWF variants found in patients with VWD can be used to properly assess defects in regulated secretion.
25010251 Therefore, VWF multimer analysis is not a valid tool to diagnose TTP in the active phase of disease but may be helpful for the diagnosis of TTP patients in remission.
24954072 vWF is actively involved in the pathophysiology of glaucoma.
24951428 Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status.
24937073 Increased VWF levels is a risk factor for cerebrovascular disease.
24790143 VWF directly binds and immobilizes extracellular DNA released from leukocytes. Therefore, we hypothesize that VWF might act as a linker for leukocyte adhesion to endothelial cells, supporting leukocyte extravasation and inflammation.
24769235 vWF is involved in the pathogenesis of HBV infection and replication, and also associated with clinicopathologic staging of HCC patients with HBV infection
24754487 The obtained results support the hypothesis of the conformational extension of VWF in shear flow.
24740645 Results support the use of FRETS-VWF73 over collagen binding assay for the assessment of ADAMTS13 severe deficiency.
24733925 The results indicate that elevation of extracellular sodium within the physiological range raises vWF sufficiently to increase coagulability and risk of thrombosis.
24700780 findings provide novel insights into VWFFVIII complex formation, leading to a greater understanding of the molecular basis of the bleeding diathesis type 2N VWD.
24681085 Flow dynamics can trigger reversible von Willebrand factor self-assembly and platelet-VWF-conglomerate accrual, which are regulated by ADAMTS13 to a time span needed by coagulation to stabilize it.
24642750 platelet vimentin engages VWF during platelet adhesion under high shear stress
24640675 In patients with hepatitis C, elevated vWF antigen levels relates to the biochemical activity of hepatitis and to a greater extent, implicates endothelial dysfunction in the pathogenesis of chronic hepatitis C.
24626470 VWF:Ag levels strongly increase upon exhaustive exercise and this increase is strongly determined by physical fitness level and the intensity of the exercise, while there is no clear effect of genetic variation in STXBP5, STX2 and the VWF promoter
24558203 VWF A2 domain stability provided by its 2 structural elements (vicinal disulphide bond and calcium-binding site) is a key protective determinant against Full Length-VWF cleavage by ADAMTS13.
24550384 Comprehensive analysis of all detected enzymes showed a random interaction mechanism for ADAMTS13 with many available binding sites on the von Willebrand factor strings
24465435 These data suggest potential race and gender divergence in regulating VWF expression by genetic variations.
24449821 We propose that plasminogen activation on endothelial cells acts as a natural backup for ADAMTS13 to degrade obstructive platelet-VWF complexes.
24441931 A high von Willebrand factor level reflects endothelial damage or dysfunction, which is a mechanism known to be involved in vascular lesions, and may promote the development of hypertensive retinopathy.
24415745 Obese children displayed higher plasma vWF than non-obese children.
24406064 OLGs provide rigidity to peptide backbones, and our findings suggest that OLG in the A1-A2 linker region regulates VWF conformational changes under shear
24394662 The shape, dimensions, and N-terminal connections of the crystal structure agree perfectly with previous electron microscopic images of VWF dimeric bouquets with the C-terminal cystine knot dimer forming a down-curved base.
24391089 These findings suggest that structural changes, including central GPIbalpha LRR-A1 contact, contribute to VWF affinity regulation.
24357063 Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent manner.
24351199 Studied whether lower VWF and higher ADAMTS13 are associated with a high postoperative drainage after CABG.
24344794 Persistently high levels of VWF is associated with insulin resisitance.
24338608 Hemodynamic forces regulate the conformation of the A2 domain and thus, its accessibility to proteolytic enzyme(s) that regulate VWF's hemostatic potential. Binding to the VWF D'D3 assemblies stabilizes coagulation factor VIII.
24327155 The F:C/vWFAg value for 90.6% of obligatory carriers was under 0.82.
24270421 vWF/p.V1316M acts directly on or downstream of Ca2+ release. This mutation is associated with severe thrombocytopathy, which likely contributes to the bleeding tendency in vWD-type 2B.
24243921 Serum thrombomodulin and von Willebrand factor levels as a parameter of endothelial dysfunction during the acute phase of acute lymphoblastic leukemia might represent an additional prognostic marker in childhood acute lymphoblastic leukemia.
24211824 Impaired kidney function affects venous thrombosis risk via concurrently raised factor VIII and von Willebrand factor levels.
24106205 platelet activation at sites of vascular injury results in the release of high local concentrations of HMWM platelet-VWF that is more resistant to ADAMTS13, thereby facilitating platelet-plug formation.
24097977 This Stx binding was localized to the A1 and A2 domain of VWF monomeric subunits and reduced the rate of ADAMTS-13-mediated cleavage of the Tyr(1605)-Met(1606) peptide bond in the A2 domain.
24075746 VWF may have a role in atherosclerosis in both the aortic arch and the carotid arteries in patients with TIA or ischemic stroke
24062306 The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet glycoprotein Ibalpha.
24056772 autophagy regulates endothelial VWF secretion, and transient pharmacological inhibition of autophagic flux may be a useful strategy to prevent thrombotic events
24014239 complement regulator factor H colocalizes with VWF in Weibel-Palade Bodies of human umbilical vein endothelial cells
24010820 VWF has a function in exocytosis that involves taking a globular conformation before expulsion from secretory pods of coalesced Weibel-Palade bodies
24010664 the size distribution of recombinant VWF and VWF-eGFP
24008159 FH contributes to the reduction of large soluble vWF multimers.
23991205 factor H binds to VWF and may modulate cleavage of VWF by ADAMTS-13
23902764 analysis of the localized dynamics-driven affinity regulation mechanism for vWF-GPIbalpha interaction
23834637 Three novel mutations of von Willebrand Factor, a potential splice site mutation (c.874+2T>C) and two frameshift mutations (c.1668delC and c.2072delCCinsG) were found.
23819767 increased affinity of p.R1306W VWF for GPIbalpha arises mostly from higher sensitivity to shear stress, which facilitates exposure of GPIbalpha binding sites
23812299 VWF represents a promising target for the treatment of cutaneous inflammation, e.g., leukocytoclastic vasculitis.
23775931 results support dual-structure/function role for the linker region between D3 and A1 domains involving a conformational equilibria that maintains quaternary A domain associations in the inactive state of vWF at low shear and an intra-A1-domain conformation that regulates the strength of platelet GP1balpha-vWF A1 domain associations in the active state of vWF at high shear
23775583 Data suggest that the amino acid substitution p.P2063S is a common neutral VWF polymorphic variant rather than a causative point mutation found only in subjects with von Willebrand disease. [LETTER]
23757730 complex formation of AnxA2 with S100A10 is a central regulatory mechanism in the acute release of VWF in response to cAMP-elevating agonists
23737952 a new function for VWF in vivo as regulator of bloodstream thrombopoiesis
23720451 Ultra large VWF contributes to the initial pathogenic step of Staphylococcus aureus-induced endocarditis in patients with an apparently intact endothelium.
23702511 mutation spectrum in 26 index cases from Turkey diagnosed with all three VWD types, the majority (73%) with parents who were knowingly related
23690449 Several common and rare VWF missense variants contribute to phenotypic differences in VWF and FVIII among African americans.
23682841 patients with severe hemophilia A have normal cleavage of VWF by ADAMTS-13 in vivo and ex vivo in the absence of FVIII
23636243 this study reports the novel demonstration that proteolytic cleavage of type 2N and HPP VWF variants by ADAMTS13 in the presence of FVIII was variably reduced as compared with that of WT.
23621778 Report mutations which seem to have different effects on the efficiency of alternative splicing, producing heterogeneous VWF variants among four patients with vone Willebrand disease 2A/IIE.
23539537 The importance of cysteine residues was determined for the structural conformation of von Willebrand factor.
23529929 These findings implicate a role for intronic splicing in mediating lineage-specific expression of vWF in the endothelium.
23520336 The presence of the D1472H sequence variation is not associated with a significant increase in bleeding symptoms, even in type 1 von Willebrand disease subjects.
23481506 The VWF-pp/ADAMTS13 ratio was associated with disease severity in patients with severe sepsis or septic shock and may help identify patients at risk for multiple organ dysfunction by detecting severe imbalance between ULVWF secretion and ADAMTS13 level.
23446343 The C2362F mutation in the B2 domain makes VWF resistant to proteolysis by ADAMTS13.
23433535 Reduction of plasma ADAMTS13 and elevation in VWF might have a role in the pathogenesis of preeclampsia.
23426949 We have used blood outgrowth endothelial cells (BOECs) isolated from human peripheral blood to analyze the storage and secretion of VWF.
23407766 Missense and deletion mutations found to be a common cause of VWD3 in cohort of Indian VWD3 patients.
23383177 The oxidative modification of von Willebrand factor is associated with thrombotic angiopathies in diabetes mellitus.
23358615 Changes of plasma vWF level in response to the improvement of air quality.
23355534 Defects in VWF mRNA levels and storage or secretion occur in type 1 VWF disease, but mRNA levels were normal in some type 2 VWF disease patients.
23354996 A common homozygous "mutation" (6187C>T; p.P2063S) in exon 36 of VWF was found in 11 families from Gujrat with severe Von Willebrand disease. The families were supposedly unrelated bu haplotype analysis suggested it was a founder mutataion.
23349392 The ratios of VWFpp/VWF:Ag and FVIII:C/VWF:Ag indicate that the pathophysiological mechanisms of type 1 VWD include reduced production and accelerated clearance of VWF, but that often a combination of both mechanisms is implicated.
23335371 Two VWF A3 domain mutations that induce a combined qualitative and quantitative defect.
23311757 In approximately 50% of Canadian families in this study the inheritance pattern for type 3 VWD is co-dominant and not recessive.
23267103 Linkage analysis identifies a locus on chromosome 2q12-2p13 for plasma von Willebrand factor that explained 19.2% of the variance in VWF levels.
23266519 VWF and ADAMTS13 might play an important role in the onset of thrombotic microangiopathy and poor outcome after living donor liver transplantation
23216583 Results highlight the complexity of VWF variations in different ethnic groups and emphasize the importance of interrogating variations on multiple ethnic backgrounds for associations with bleeding and thrombosis.
23179108 Report mutations in type 2 von Willebrand disease.
23168412 the N terminus of VWF is critical for the interaction with FVIII and Ser-764 and Lys-773 have opposite roles in the binding mechanism
23104847 Report von Willebrand factor elongation and fiber formation on collagen during acute whole blood exposure to pathological flow.
23041092 Both von Willebrand factor and its cleaving protease ADAMTS13 play an important role in atrial fibrillation.
23020229 This study was conducted to determine the effects of smoking on vWF and ADAMTS-13 antigen and activity levels in Arab males.
23006049 VWF interaction with glycoprotein Ib is modified by polyphosphate
22982001 VWF SNPs rs980130,216809, 4764478, 2885517, & 12580343 correlated with subclinical carotid atherosclerotic plaque. vWF may play a role in carotid plaque burden/rupture by modifying coagulation & inflammatory responses toatherosclerosis.
22972845 Data suggest that expression of vWF and endothelin-1 is up-regulated and eNOS (endothelial nitric oxide synthase) activity is increased in radial artery in diabetic patients with coronary artery disease.
22952809 PAR signaling and serine protease-induced alterations in endothelial function modulate glomerular inflammation via parallel but independent pathways
22923007 The vWF gene polymorphisms at site A1381T were not associated with coronary heart disease, but plasma vWF levels were influenced by vWF gene polymorphisms at site A1381T, blood type and coronary heart disease.
22922961 Data indicate that exposure of von Willebrand factor sites for glycoprotein Ibalpha binding and ADAMTS13 cleavage are coupled.
22896002 High VWF is associated with Waldenstrom macroglobulinemia.
22871923 explored genotype and phenotype characteristics of patients with VWD with the aim of dissecting the distribution of mutations in different types of VWD
22854959 Light chain of factor VIII is sufficient for accelerating cleavage of von Willebrand factor by ADAMTS13 metalloprotease.
22849435 H antigen is not restricted to particular N-glycosylation sites. Also, the Asn(2635) site, previously designated as unoccupied, was found to be highly glycosylated
22812646 investigated the effect of shear stress on the uptake of FVIII-GFP in presence of VWF. Immunofluorescence studies demonstrated that VWF does not block endocytosis of FVIII-GFP under flow conditions
22781599 The A1500E mutation resulted in VWF more susceptible to ADAMTS13-dependent proteolysis.
22740102 anti-von Willebrand factor aptamer ARC1779 increases von Willebrand factor levels and platelet counts in patients with type 2B von Willebrand disease
22718838 Stx1B and Stx2B induce acute VWF secretion in a PLD1-dependent manner but do so by differentially modulating PKCalpha, RhoA, and ADP-ribosylation factor 6.
22688555 vWF promoter polymorphisms influence corticosteroid-mediated increase in vWF especially in patients with haplotype 1.
22674667 Fourteen different mutations scattered throughout the VWF gene were identified, 10 of which were novel.
22612323 The presence of vWF in the maternal space and its diminished expression in syncytiotrophoblast of pre-eclamptic placenta also might have pathogenic implications.
22596213 Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis.
22593709 Results indicate that there might be no correlation between pseudoexfoliation syndrome (PEX) and such endothelial cell markers as von Willebrand antigen (vWF Ag), E-selectin, and P-selectin concentrations.
22568520 findings therefore do not support a strong causal relationship between VWF and CHD in elderly individuals of >/= 55 years, but suggest that VWF is primarily a marker of CHD
22559004 The data indicated that the interaction between factor VIII and von Willebrand factor is mediated mainly by electrostatic forces, and that it is not accompanied by entropic constraints.
22545467 The increase of the level of endothelin-1 and the activity of von Willebrand factor is a risk factor of the development of pulmonary hypertension in children with chronic bronchopulmonary pathology.
22537243 findings have established for the first time that I1416N and I1416T are responsible for a type 2M VWD phenotype and demonstrate that quantification of VWF function under shear stress may provide an accurate measure of clinical severity
22532296 vWF-Ag is a new, simple and noninvasive predictor of Clinicall significant portal hypertension.
22517896 These data demonstrate an important functional role of VWF O-linked glycans under shear stress conditions in platelets.
22507806 Subjects with O blood group have 25% less VWF than those of non O blood groups, and the latter show higher thrombus burden.
22507569 VWF:CB may be useful in diagnosis of VWD, as a decreased VWF:CB/VWF:Ag ratio may reflect loss of collagen binding ability. Mutations that exclusively affect type VI collagen binding may be associated with bleeding, yet missed by current VWF testing
22503077 analysis of VWF gene expression showed that c.7674-7675insC mutation in VWF gene resulted in degradation of VWF mRNA via NMD.
22490677 Homologies of domains in VWF to domains in other proteins allow many disulfide bonds to be tentatively assigned, which may have functional implications.
22487084 Report differential sensitivities and reproducibility of von Willenbrand factor activity assays.
22479377 study identified a unique VWF mutation responsible for the bleeding phenotype in a patient family with type 2A VWD; the mutation impaired VWF trafficking through the endoplasmic reticulum, thereby preventing VWF secretion from the cells
22477538 This review provides an overview of recent findings on the role of VWF in hemorrhagic and thrombotic conditions. [review]
22466813 Relationship between ABO blood groups and von Willebrand factor, ADAMTS13 and factor VIII in patients undergoing hemodialysis.
22452980 Von Willebrand factor (VWF) propeptide binding to VWF D'D3 domain attenuates platelet activation and adhesion.
22431729 N-terminal flanking region of A1 domain in von Willebrand factor stabilizes structure of A1A2A3 complex and modulates platelet activation under shear stress.
22431572 Type 2A VWD appears to result from a complex intersection of mechanisms that include: (1) intracellular retention or degradation of VWF, (2) defective multimerization, (3) loss of regulated storage, and (4) increased proteolysis by ADAMTS13.
22429825 Homozygous/heterozygous quantitative VWF deficiency caused by missense VWF mutations in different VWF domains can be associated with inability to form endothelial Weibel-Palade-like granules.
22370688 The discovery of ADAMTS13 has provided a critical impetus for understanding the regulation of von Willebrand factor activity by shear stress[review]
22357761 VWF binds calcium at physiologic calcium concentrations and calcium stabilizes VWF A2 by accelerating refolding
22298244 The imbalance between ADAMTS13 and VWF levels does not explain the development of VAT in HD patients by itself, although it should contribute for the hypercoagulability state.
22282355 ARC15105 is a potent antagonist of von Willebrand factor mediated platelet activation and adhesion.
22268915 Letter: a high VWFpropeptide/VWF:antigen ratio characterizes patients with a first acute episode of TTP, reflecting a potential association with disease severity.
22267483 Have identified galectin-1 and galectin-3 as novel partners for VWF, and these proteins may modulate VWF-mediated thrombus formation.
22257027 Eight SNPs that strongly determine VWF levels are not associated with stroke risk, either individually, or combined in a genetic score.
22244714 Genomic analysis of various hemostatic factors identified von Willebrand factor as a potential predictor of hematoma expansion in patients with intracerebral hemorrhage.
22219226 Data show that four intronic Factor VIII (FVIII) single nucleotide polymorphisms (SNPs) associated with FVIII activity and 8 with FVIII-VWF (von Willebrand factor) ratio in a sex- and race-dependent manner.
22207689 Mutations of cysteines forming intrachain disulfide bonds lead to reduced von Willebrand factor storage and secretion because the von Willebrand factor is retained in the endoplasmic reticulum.
22197721 Results indicate that the sequence variations common in the African American population are rare in whites and may have been misidentified as VWF mutations.
22178067 Our results indicate a shear- and platelet-dependent role for several proteases in the local modulation of the VWF function.
22163125 High von Willebrand factor level is related to the no-reflow phenomenon after primary percutaneous coronary intervention in such a way that it might be a predictor of the phenomenon.
22125593 Data show that median VWF and propeptide levels were significantly higher in patients with uncomplicated malaria than in children with non-malarial febrile illness of comparable severity, in whom levels were higher than in non-febrile controls.
22110247 Data suggest that high VWF and low ADAMTS13 plasma levels both increase the risk of ischemic stroke and myocardial infarction, and the risks associated with high VWF or low ADAMTS13 levels are further increased by the use of oral contraceptives.
22100896 CD40 ligand, soluble P-selectin and von Willebrand factor may ahve a role in prediabetes
22096550 Data show temperature-dependence of Weibel-Palade body exocytosis and cell surface dispersal of von Willebrand factor and its propolypeptide.
22095559 HIV-1 gp120 downregulates vWF expression in human mesenchymal stem cells
22091998 oxidation of Met1606, impairing ADAMTS-13 cleavage, results in the accumulation of vWF polymers, which recruit and activate platelets and bind tightly to bacterial adhesins, contributing to the development of thrombotic and septic complications in CKD.
22077817 a significant decrease in the bleeding time after a tail-clip challenge in mice treated with plasmids expressing hFVIII and phiC31 integrase
22070827 Findings suggest that ADAMTS13/VWF profiles may have important roles in the pathogenesis of DIC.
22056526 Our experiments provide evidences that neutralizing the acid and adding NaCl in physiological concentration, thereby facilitating formation of collagen fibril molecules in solution, results in efficient coating of human type-I and type III collagens.
22032085 The correlation between each pairs of coagulation factors, FV, FVIII and vWF could be easily interpreted using previously characterized interactions between these factors.
22004444 study describe a family with normal VWF:RCo (von Willebrand factor ristocetin cofactor activity) but reduced VWF:CB and a M1761K mutation in the A3 domain
21940821 Intra-cellular and extracellular assembly of FVIII-VWF complex involves distinct mechanisms, which differ with regard to their implications for platelet binding to released VWF strings.
21937160 variable effects that chemical modifications/mutations in vWF have on proteolysis by ADAMTS13
21917758 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
21911836 lateral VWF dimers form when a Cys2431 thiolate anion attacks the Cys2431 sulfur atom of the Cys2431-Cys2453 disulfide bond of another VWF molecule, whereas the Cys2451-Cys2468 disulfide/dithiol mediates formation of trimers and higher-order oligomers
21909423 GrM also exists extracellularly in plasma where it could play a physiological role in controlling blood coagulation by determining plasma FVIII levels via proteolytic processing of its carrier VWF.
21904649 Acute coronary syndromes patients had significantly higher vWf activity and vWf antigen levels, as well as TBARS levels, while their levels of NO(2)-, H2O2, SOD and CAT activities were lower than controls'.
21896483 Local elongation of endothelial cell-anchored von Willebrand factor strings precedes ADAMTS13 protein-mediated proteolysis.
21857647 analysis of a pH-regulated dimeric bouquet in the structure of von Willebrand factor
21847508 The differential immunoexpression of vWF at different tissue types of preeclamptic placenta and endothelial dysfunction markers at maternal serum of preeclamptic pregnancies, were examined.
21844207 Endothelial cell actomyosin II filament ring associates with exocytosing Weibel-Palade bodies to expel von Willebrand factor.
21839029 REVIEW OF THE ROLE OF VWF IN HEMOSTASIS, THROMBOSIS, BLEEDING
21831097 Report value assignment of the WHO 2nd International Standard von Willebrand factor, concentrate (09/182).
21810271 study generated knowledge on genomic regions deserving further investigation in search for genetic factors influencing vWF and FVIII plasma levels, some potentially implicated in venous thrombosis
21792464 Therefore VWD and TTP represent the opposite manifestations of VWF related disorders, tightly linked to each other
21779388 Data show that an anti-VWF mAb against the VWF-A2 domain (A1555-G1595) reduces the proteolytic cleavage of VWF by ADAMTS13 under shear stress, suggesting the role of this region in interaction with ADAMTS13.
21729570 Plasma vWF level in vWF gene A1381T polymorphism with AG mutant was significantly higher than GG mutant in coronary heart disease patients.
21715306 Protease activity of ADAMTS13 is controlled by conformational changes in its substrate, von Willebrand factor (VWF), which are induced when VWF is subject to elevated rheologic shear forces. (Review)
21711445 Fourteen VWF mutations were identified in 17 of 23 index cases (aged 5-17 years), including four that were previously unreported (L60P, nt1658 insT, Q1388X, and C2237F).
21705658 The recognition of VWF Leu1603 by ADAMTS13, in conjunction with previously reported remote exosites C terminal of the cleavage site, suggests a mechanism whereby the VWF P1-P1' scissile bond is brought into position over the active site for cleavage.
21638248 The post-carotid artery stenting levels of P-selectin, vWF and endothelin-1 are related to the extent of endothelial injury.
21629201 vWf seems to be profoundly related to platelet volume in subjects with isolated impaired fasting glucose.
21612582 HIV-1 gp120 downregulates vWF expression in human mesenchymal stem cells
21605335 Oxidized von Willebrand factor multimers are cleaved by leukocyte serine proteases, under conditions where high concentrations of oxidant species would severely reduce the proteolytic efficiency of ADAMTS-13.
21596755 defects in the intracellular storage and regulated secretion of VWF following ER retention may be a common mechanism underlying VWD with a quantitative deficiency of VWF.
21592973 pH sensing by evolutionarily conserved His residues facilitates the assembly and packaging of VWF multimers upon arrival in the trans-Golgi
21592258 A novel heterozygous missense mutation in the A1 domain of Von Willebrand Factor gene responsible for type 2A phenotype. Exon 28 of VWF gene showed a 3815 G --> T transversion resulting in C1272F mutation.
21518247 found no evidence to support a relationship between genetic variations in thrombospondin genes and the occurrence of CHD; genetic variations in THBS1 and THBS2 are weakly involved in the regulation of VWF:Ag
21512165 Preeclampsia is associated with decreased levels of ADAMTS13, independently of von Willebrand factor.
21507943 GP Ibbeta/GP IX mediates the disulfide-linked GP Ibalpha localization to the GEMs, which is critical for vWf interaction at high shear
21474661 Report differential roles of fibrinogen and von Willebrand factor on clot formation and platelet adhesion in reconstituted and immune thrombocytopenia.
21471547 Data show that the baseline levels of both vWF and sES were higher in the patients with PHPT, while at the 6-month follow-up, vWF decreased significantly and sES was normal.
21410641 analysis of deletions in VWF identified in patients with von Willebrand disease using multiple ligation-dependent probe amplification
21393328 This is the first report of a synonymous von Willebrand factor substitution (7056C>T)being responsible for von Willebrand disease.
21362127 A large novel deletion in VWF, most probably due to a founder effect, seems to be unique to Hungarian type 3 VWD patients with high allele frequency
21352469 the aortic pressure gradient is inversely associated with VWF activation factor, but not with VWF antigen or VWF multimerization in patients with aortic stenosis
21346256 In vitro biosynthesis was reduced for both R1205H and Y1584C mutations in human and mouse VWF, with the effect being more severe in R1205H.
21343614 we identified a cluster of intronic von Willebrand factor (VWF) single nucleotide polymorphisms that associate with plasma levels of VWF
21342431 effect of genetic variability in the VWF gene (VWF) on VWF antigen and activity levels, and on the risk of cardiovascular disease (Review)
21304226 results indicate that the cortisol-induced increase in VWF may be predicted by VWF promoter polymorphisms, haplotype 1 and (GT)(S) being the sensitive pattern.
21266777 Data show that N-acetylcysteine reduced soluble plasma-type von Willebrand factor (vWF) multimers in a concentration-dependent manner and rapidly degraded ultra-large vWF multimer strings extruded from activated endothelial cells.
21174003 von Willebrand factor is inhibited by ARC1779 in patients with acute thrombotic thrombocytopenic purpura
21163921 Genetic variation in VWF gene is associated with venous thrombosis.
21150180 Serum TM and vWF levels might represent additional, but not independent, prognostic markers in childhood ALL.
21143374 association between SNP c.- 2527G>A, the (GT)n short tandem repeat and VWF:Ag levels in healthy controls
21136020 von Willebrand factor and ADAMTS13 have a role in clinical outcome after cardioversion for atrial fibrillation
21094987 Have established a simple and reliable method to detect decreased binding of FVIII to von Willebrand factor in plasma samples.
21083900 Activation of p38 MAPK in endothelial cells by von Willebrand factor is responsible for the regulation of endothelial cell adhesiveness for mesenchymal stem cells.
21048155 Studies identify a new function of VWF in ECs, which confirms VWF as a protein with multiple vascular roles and defines a novel link between hemostasis and angiogenesis.
21044367 Observational study of gene-disease association. (HuGE Navigator)
21037087 -induced activation of GpIb-V-IX triggers platelet procoagulant activity and anchorage of a star-like fibrin network.
21029358 Letter: report impact of newly discovered genetic variations on FVIII and VWF plasma levels and venous thrombosis risk.
20979592 thiol-dependent interaction of beta(2) GPI with VWF may contribute to the redox regulation of platelet adhesion
20958794 E2 may play a role in the regulation of VWF and ADAMTS13 gene expression and in its production in human endothelial cells
20946172 novel disulfide-bond-reducing activity of ADAMTS-13 may prevent covalent lateral association and increased platelet adherence of plasma-type VWF multimers induced by high fluid shear stress
20941784 geetic association study of functional consequeces of VWF variations
20940418 Common variants in the VWF gene are associated with VWF levels and with the risk for cardiovascular diseases.
20940418 Observational study of gene-disease association. (HuGE Navigator)
20854374 identification of the genetic basis of VWD in three patients confirmed that mutations leading to null alleles in the VWF gene are associated with allele-specific mRNA degradation
20851871 Studies indicate that VWF SNP within primer annealing sites complicate molecular analysis of VWD, specifically detection of heterozygous mutations.
20806123 Report on the evaluation of seven commercial von Willebrand factor collagen binding assays to potentially assist the discrimination of types 1 and 2 von Willebrand disease.
20806106 higher levels in cirrhotic patients
20737318 vWF in human colorectal cancer cells plays an important role in promoting proliferation, adhesion, and migration.
20729548 Collagen VI microfibril formation is abolished by an {alpha}2(VI) von Willebrand factor type A domain mutation in a patient with Ullrich congenital muscular dystrophy.
20725043 demonstration of two states of the receptor-ligand bond, that is, a flex-bond--between the A1 domain of VWF and the glycoprotein Ib alpha subunit
20723310 Plasma vWF level is unrelated to sex but interrelates with ABO blood groups.
20723310 Observational study of gene-disease association. (HuGE Navigator)
20705333 ADAMTS13 interacted with the rVWF A1, A2, A3 domains and full-length VWF, while TSP1 also bound to three A domains, especially to A2 and A3 domains.
20704649 von Willebrand factor clearance does not involve proteolysis by ADAMTS-13
20701764 removal of the N-terminal part of full length AgRP and addition of a VWF signal peptide is a successful strategy to deliver neuropeptide minigenes to the brain
20696945 The 13-bp deletion mutation alters the binding of Ets (and possibly GATA) proteins to the VWF promoter and significantly reduces VWF expression.
20696943 Fluid shear in circulation can increase the effective size of VWF bound to platelet GpIbalpha via protein self-association. This can trigger mechanotransduction and cell activation by enhancing the drag force applied on the cell-surface receptor.
20695979 ADAMTS13 CUB and T2-8 domains influence proteolysis of platelet-decorated VWF strings in vivo
20682599 change ADAMTS13 activity might modulate the risk of DVT by altering vWF and FVIII levels, the polymorphisms analyzed in this study did not correlate with DVT risk among patients investigated.
20682599 Observational study of gene-disease association. (HuGE Navigator)
20675279 Data show no correlation between von Willebrand Factor, CD31, and CD34 and glomerular segmental sclerosis, interstitial fibrosis, activity and chronicity, which suggests a loss of these markers and microvasculature involvement.
20673868 Observational study of gene-disease association. (HuGE Navigator)
20651279 Men who slept for short and long durations had higher vWF levels. In women, there was a significant nonlinear association.
20650506 Evaluate the diagnostic efficiency of a rapid quantitative latex immunoassay test for the measurement of VWF antigen.
20628086 Observational study of gene-disease association, gene-environment interaction, and pharmacogenomic / toxicogenomic. (HuGE Navigator)
20627205 FVIII, VWF and Fg determinations should be accompanied by hsCRP measurement to assess an inflammatory response that may potentially mask deficiency states or mimic an increased risk of thrombosis.
20605782 FVIII and platelets are cofactors that regulate proteolysis of multimeric VWF by ADAMTS13 under physiological conditions.
20589320 mRNA levels of CD31, CD144, CD146 and vWf in whole blood as detected by real time RT-PCR cannot be used as biomarkers for end-stage endothelial cells such as circulating endothelial cells.
20589314 Tapping mode atomic force microscopy show the overall structure of recombinant VWF is similar to that of plasma derived VWF and that rVWF will extend its conformation under shear stress, which is required to exert its function in primary haemostasis.
20589313 ARC1779 is a potent inhibitor of the enhanced VWF function in VWF type 2B patients.
20586924 a homozygous R854W mutation in the D' domain of VWF induces impaired secretion and activity of the protein, thereby explaining the severe phenotype of the patient
20570857 Coexpression of the wild-type and in-frame deleted (p.P1127_C1948delinsR) VWF forms impaired protein secretion, high molecular weight multimer formation and function
20565774 Observational study of genotype prevalence. (HuGE Navigator)
20561182 Blood group significantly influences von Willebrand factor increase and half-life after desmopressin in von Willebrand disease Vicenza
20508517 Increased serum vWF and sVCAM-1 levels are associated with late stent thrombosis (ST), suggesting that endothelial dysfunction contributes to the development of late or very late ST.
20508517 Observational study of gene-disease association. (HuGE Navigator)
20505748 a direct thrombin-independent response of endothelial cells (ECs) to MV3 melanoma-derived supernatant was measured by rapid release of VWF
20498367 Termination of A domain association within VWF in solution results in binding to GPIba and platelet activation under high shear stress.
20492463 von Willebrand factor variant p.Arg924Gln marks an allele associated with reduced von Willebrand factor and factor VIII levels
20492463 Observational study of gene-disease association. (HuGE Navigator)
20491956 alanine or threonine 789 to proline mutation causing type 2N von Willebrand's disease
20452482 Observational study of gene-disease association. (HuGE Navigator)
20431063 NFIB/X and NFY repressors contribute differentially to organ-specific regulation of the VWF promoter in transgenic mice.
20424473 Observational study of gene-disease association. (HuGE Navigator)
20418283 Further evidence of the critical role that individual N-linked glycans play in determining VWF synthesis and expression.
20403097 The 12E-V2R variant has increased binding affinity for AVP, resulting in increased signal transduction, and is associated with increased levels of VWF propeptide, VWF, and FVIII.
20354169 Mutagenesis of C1669/C1670 in full-length VWF resulted in markedly increased susceptibility to cleavage by ADAMTS13, confirming the important role of the paired vicinal cysteines in VWF A2 domain stabilization.
20351307 A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE.
20346360 Observational study of gene-disease association. (HuGE Navigator)
20335223 identified an N528S homozygous mutation in the VWF propeptide D2 domain, predicting the introduction of an additional N-glycosylation site at amino acid 526 in close vicinity to a "CGLC" disulphide isomerase consensus sequence.
20303469 The shorter VWF half-life found in patients with no VWF mutations suggests that mechanisms other than VWF might be involved in the pathogenesis of type 1 von Willebrand disease.
20231535 New genetic associations were discovered outside previously known biological pathways. For VWF, 400 SNPs exceeded the genome-wide significance threshold and marked 8 loci on 6 chromosomes
20231535 Observational study, meta-analysis, and genome-wide association study of gene-disease association. (HuGE Navigator)
20231421 Our results suggest that racial differences in VWF ristocetin cofactor:VWF antigen activity ratios are the result of increased frequency of the D1472H polymorphism in African Americans.
20231421 Observational study of gene-disease association. (HuGE Navigator)
20218000 Study compares selected properties of purified tryptic fragments of human von Willebrand (vW) protein in order to better understand how the M(r) 116,000 fragment retains activity in ristocetin-induced platelet agglutination.
20201926 Observational study and genome-wide association study of gene-disease association. (HuGE Navigator)
20197612 HIF-1alpha in coordination with vWF may play an important role in the pathogenesis of pre-eclampsia.
20156642 SNPs haplotypes in the VWF gene promoter, but also the variable-length (GT)(n) locus predict the risk of developing high VWF levels in Cushing's syndrome under conditions of glucocorticoid excess
20142117 Simvastatin and ezetimibe did not alter von Willebrand factor levels in patients with hypercholesterolemia.
20118404 Patients with VWD2B with 7 different gain-of-function mutations had some structurally abnormal giant platelets. Megakaryocytopoiesis is modified by the enhanced VWF-GPIbalpha interactions.
20075158 Site-directed mutagenesis, kinetic analyses, and peptide inhibition assays have identified a role for amino acid residues Arg(659), Arg(660), and Tyr(661) of ADAMTS13 in proteolytic cleavage of various substrates.
20063990 Data suggest that integrin alpha(2)beta(1), glycoprotein Ib and vWf interactions with collagen I and III contribute to platelet adhesion under high shear flow.
20062916 Results suggest that decreased ADAMTS13 activity in combination with increased VWF concentrations may contribute to the complications in severe malaria.
20060388 The coamplification of VWF pseudogene is clearly a major impediment to genetic diagnosis of VWD.
20058209 propensity of VWF and platelet to form microvascular thrombosis is mitigated by ADAMTS13, which cleaves VWF before it is activated by shear stress to cause platelet aggregation in the circulation
20036902 Observational study of gene-disease association. (HuGE Navigator)
20032502 An autoantibody epitope comprising residues R660, Y661, and Y665 in the ADAMTS13 spacer domain identifies a binding site for the A2 domain of VWF.
20030949 DXS15, CA13 and CA22 are highly polymorphic genetic markers useful for linkage analysis of haemophilia A.
20024497 VWF protects fibrinogen against degradation by plasmin, preserving its clottability in plasma and its adhesive role in platelet-rich thrombi.
20021313 Baseline von Willebrand factor and circulating endothelial cell levels were higher in patients with impaired glucose tolerance vs those with normal glucose tolerance and healthy controls
19965639 Sialic acid protects VWF from proteolysis by Ser & Cys proteases but specifically enhances ADAMTS13 susceptibility. Quantitative variation in VWF sialylation is a key determinant of VWF multimeric composition & may have pathophysiologic significance.
19948975 Observational study of gene-disease association, gene-gene interaction, gene-environment interaction, and genetic testing. (HuGE Navigator)
19944670 two epitope sites for VWF binding in the spacer domain of ADAMTS13 using the lambda-phage surface display system were explored.
19943880 Suggest that reduced affinity Cl binding/increased susceptibility toADAMTS-13 cleavage might be a new common feature of VWD2B variants.
19933990 Observational study of gene-disease association and gene-gene interaction. (HuGE Navigator)
19913121 Observational study of gene-disease association. (HuGE Navigator)
19897584 Force-induced cleavage of single VWFA1A2A3 tridomains by ADAMTS-13.
19897581 Plasmodium falciparum infected erythrocytes adhere via platelet decorated ultra-large VWF strings on activated endothelium
19875727 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.
19874459 To define an exhaustive profile of the VWF O-glycan structures to help the understanding of their role in VWF regulation and properties.
19840363 GPIbalpha-VWF interaction induces apoptotic events in platelets, and that the association of 14-3-3zeta with the cytoplasmic domain of GPIbalpha is essential for apoptotic signaling.
19839997 Plasma levels of sTNF-R1 and vWf were statistically significantly increased in both bipolar disorder and schizophrenia compared to control and were also increased in unmedicated patients.
19820200 Shear-induced interaction of platelets with von Willebrand factor results in glycoprotein Ibalpha shedding.
19817991 tension in VWF due to shear flow selectively exposes the A2 proteolysis site to ADAMTS13 for cleavage while keeping the folded remainder of A2 intact and functional
19812385 Reactive oxygen species released by activated neutrophils have a prothrombotic effect, mediated in part by inhibition of VWF cleavage by ADAMTS13.
19773258 Premature termination codon mutations in the von Willebrand factor gene are associated with allele-specific and position-dependent mRNA decay.
19765213 initial activation-independent platelet adhesion to VWF via GPIb is resistant to NO, however, NO inhibits GPIb-mediated activation of alpha(IIb)beta(3) and MLC leading to reduced platelet spreading and aggregation.
19765212 ADAMTS-13 binds to circulating VWF and may therefore be incorporated into a platelet-rich thrombus, where it can immediately cleave VWF that is unfolded by fluid shear stress.
19740526 A family having type 2B von Willebrand disease with an RW1306W mutation is described. Severe thrombocytopenia leads to normalization of high molecular weight multimers.
19732980 Plasma levels of von Willebrand factor were significantly higher in patients with no-reflow phenomenon.
19730683 Observational study of gene-disease association. (HuGE Navigator)
19729451 ADAMTS13 bound to endothelial cells and enhanced cleavage of VWF.
19726719 The structure of a complex between GpIbalpha and the cyclic peptide inhibitor Callosteric TERMALHNLC defines a previously unrecognized VWF binding site within GpIbalpha.
19718479 von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity may have roles in chronic heart failure
19718474 a G-quartet oligonucleotide specifically blocks VWF binding to GP Ibalpha
19687512 mutations causing an abnormality in the binding of VWF to collagen may contribute to clinically significant bleeding symptoms. We propose that isolated collagen-binding defects are classified as a distinct subtype of von Willebrand disease.
19682236 Incubation of the histamine-stimulated endothelial cells with recombinant ADAMTS13, regardless of flow, did not alter the VWF multimer distribution.
19647361 VWF multimers form supramolecular structures favoring platelet trapping in solution under static conditions when the conformational state of the protein is changed only by chemical potential of allosteric effectors
19630816 upregulated VWF function can lead to abnormal VWF trafficking in megakaryocytes, a modified platelet production with interacting proplatelets, and the presence or even release of platelet agglutinates in the bone marrow [REVIEW]
19630761 self-organizing behaviors of VWF in cell compartments [review]
19624459 The frequency of R924Q in the normal & type 1 VWD populations was ascertained. It does not affect biosynthesis, intracellular trafficking or storage. A new truncated transcript results from activation of an exon 28 cryptic splice site.
19624459 Observational study of gene-disease association. (HuGE Navigator)
19608674 results suggest that a complete absence of VWF (VWD3), as well as enhanced VWF binding to GPIb (VWD2B), may interfere with megakaryocytopoiesis
19587373 This novel VWF C-terminal binding site may participate as the initial step of a multistep interaction ultimately leading to proteolysis of VWF by ADAMTS13.
19570985 VEGFR2, but not VEGFR1, is responsible for the release of von Willebrand factor.
19566550 The pathogenic nature of 11 of 14 candidate missense mutations identified in patients with type 1 VWD was confirmed. Intracellular retention of mutant VWF is the predominant responsible mechanism
19541819 Activated leukocytes may participate in the proteolytic regulation of VWF.
19506362 Increased proteolysis due to mutations in the A2 domain exon 28 leads to VWD type 2A, with the absence of high, intermediate & large VWF multimers, pronounced triplet structure of individual bands & increased VWF degradation products. Review.
19506361 VWF multimeric analysis in VWD 2M & 2U (due to a loss-of-function mutation in the A1 domain) show decreases in large VWF multimers with less resolved triplet structure of each of the multimeric bands in low-, medium- or high-resolution gels. Review.
19506360 In type 2M of VWD, although VWF-platelet interaction is altered, high-molecular-weight multimers are present so that the von Willebrand factor (VWF):collagen binding VWF:Ag ratio is normal. Review.
19506359 A new set of missense mutations in D4, B1-B3 and C1-C2 domains has been discovered as the cause of a mild VWD type 1 secretion defect with normal VWF multimers or smeary VWF multimeric pattern. Review.
19506358 Recessive VWD type Normandy is due to mutations involving exons 18-21 or 24, affecting binding of VWF to Factor VIII, resulting in loss of FVIII:C activity and hemophilia. Review.
19506357 Recessive VWD type 2C (2A subtype IIC) is caused by homozygosity or double heterozygosity of missense mutations in the D1 & D2 domains of the VWF propeptide (pp) that catalyzes the multimerization in the D3 domain at the N terminus of mature VWF. Review.
19506356 Some autosomal recessive VWF homo- or compound heterozygotes produce patterns from absence of HMW multimers to the presence of multimer forms lacking triplet structure, with faster-running central band, smeary structure & indistinct satellites. Review.
19506355 Genetic, molecular & clinical aspects of von Willebrand disease (VWD) type 2N & of mild hemophilia A due to mutations impairing FVIII-von Willebrand factor (VWF) interactions are reviewed. Review.
19506354 The C1584 variant is associated with decreased VWF levels & function, and with slightly enhanced VWF proteolysis & clearance. Review.
19506353 The regions of VWF affected by mutations in type 1 von Willebrand disease are described and classified. Review.
19506352 The structure of VWF and its genes and mutations are reviewed. Review.
19498171 findings demonstrate that elongational forces in the range experienced by VWF in the vasculature unfold the A2 domain, and only the unfolded A2 domain is cleaved by ADAMTS13
19496923 A region of the VWF promoter between -2182 and the end of the first intron contains information for LPS-mediated gene repression
19477487 vWF levels were significantly elevated in end-stage renal disease patients compared to controls
19470641 The 1.9-A crystal structure of the A2 domain demonstrates evolutionary adaptations to this shear sensor function. Unique among VWF A (VWA) domains, A2 contains a loop in place of the alpha4 helix, and a cis-proline.
19436051 significantly increased expression of EPCR and TM in the valvular sinus endothelium as opposed to the vein lumenal endothelium, and the opposite pattern with VWF
19422453 The propositus phenotype seems to be due to mutations R202W and C849Y, both affecting the VWF multimerization process and, for C849Y rVWF, intracellular survival
19422343 Development of a hyperreactive primary hemostatic system, as evidenced by high levels of fully functional VWF and a temporary ADAMTS13 deficiency during liver transplantation.
19420105 Observational study of gene-disease association. (HuGE Navigator)
19372260 A new a deletion (c.221-977_532 + 7059del [p.Asp75_Gly178del]) was found in 7 of 12 white type 3 VWD patients from 6 unrelated families. Expression studies indicated markedly decreased secretion and defective multimerization of the mutant VWF protein.
19350125 Case Report: Heyde's syndrome without a decrease in large von Willebrand factor multimers: a case of intestinal bleedings reversed by valve replacement in a patient with aortic stenosis.
19346881 We have identified a previously unreported mutation, V1499E, with a high penetrance in a family with type 2A von Willebrand disease
19298374 Observational study of genetic testing. (HuGE Navigator)
19289057 Changes in von Willebrand Factor conformation reported here likely regulate protein function in response to fluid shear.
19286880 Data provide evidence that for VWD classification factors other than basal VWF, such as DDAVP response and platelet VWF, should be considered.
19277422 molecular diagnosis of von Willebrand disease by direct sequencing; 30 VWF variations were identified; 12 of these are new, including 4 missense, 1 nonsense, 1 insertion, the first insertion-deletion described in VWF & 5 potential splice site mutations
19192112 demonstrate reduced secretion, loss of VWF multimerization, and defective regulated storage of a variant VWF
19190814 Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia.
19190813 Microsatellite (GT)(n) repeats and SNPs in the von Willebrand factor gene promoter do not influence circulating von Willebrand factor levels under normal conditions.
19190813 Observational study of gene-disease association. (HuGE Navigator)
19190805 Variations in the ratio between von Willebrand factor and its cleaving protease, ADAMTS13, during systemic inflammation and association with severity and prognosis of organ failure.
19175495 MMRN1 supports platelet adhesion by VWF-dependent and -independent mechanisms that vary by flow rate
19110358 Markers of inflammation (factor VIIIc, von Willebrand factor, white blood cell count) and hemostasis are associated with greater risk of kidney function decrease.
19088379 VWF type 2N variants are still capable of cotargeting FVIII to storage granules implies that trafficking of Weibel-Palade bodies cargo proteins does not necessarily require high-affinity assembly with VWF.
19060241 In reevaluating this kindred, we determined that patients with Montreal platelet syndrome have type 2B von Willebrand disease with the V1316M VWF mutation.
19029465 Higher levels of vWF were associated with risk of CVD in people with type 2 diabetes mellitus or insulin resistance, which suggests that vWF may be a risk factor unique to these populations.
19021456 The Msp I polymorphism in intron 19 (M+ allele) of vWF(vonWillebrand Factor) gene is associated with the development and the severity of preeclampsia
19021456 Observational study of gene-disease association. (HuGE Navigator)
18996572 Findings suggest that the imbalance between VWF and ADAMTS13 levels caused by atrial remodeling might be closely associated with intra-atrial thrombus formation in atrial fibrillation patients.
18983500 The rapid expulsion and unfolding of long VWF strings that remain anchored to endothelial cells after secretion from Weibel-Palade bodies can alter the structure or exposure of some VWF monomeric A2 domains to create an ADAMTS-13-cleavable form.
18983499 inhibitory effect of VWF added decreased with decreasing rFVIII concentration in FVIII/VWF-depleted substrate plasma
18981290 After its secretion, ADAMTS13 does not require N-glycans for its VWF cleaving activity
18945966 different mechanisms appear to control the agonist-induced secretion of VWF and tissue-type plasminogen activator, with only the former requiring phospholipase D1
18927433 VWF strings bind specifically to integrin alpha(v)beta(3) on human endothelial cells
18923835 789Ala/Ala genotype was found significantly higher in quantitative type (type 1 and type 3) VWD that is occurred due to low VWF:Ag level.
18923835 Observational study of gene-disease association. (HuGE Navigator)
18855616 The present review illustrates the dynamic processes involved in the functional cross-talk between von Willebrand factor and thrombospondin-1, both proteins having properties that are highly controlled by the shear stress in their environment.
18854154 Knockdown of von Willebrand factor (VWF) by siRNA inhibits the early stages of HIV-1 replication in 293T cells infected with VSV-G pseudotyped HIV-1
18848323 Observational study of gene-disease association. (HuGE Navigator)
18841300 VWF R924Q polymorphism could indicate factor VIII binding defect
18809794 In obstructive hypertrophic cardiomyopathy, VWF impairment is frequent and is closely and independently related to the magnitude of outflow obstruction
18776767 an observed G to C mutation at base 3923 of the VWF gene was previously described in a family with circulating platelet clumps and abnormal megakaryopoiesis with release of clumped platelets in culture
18771467 Elevation of serum von Willebrand factor and anti-endothelial cell antibodies in patients with immunoglobulin A nephropathy are associated with intrarenal arterial lesions.
18761720 VWF remained as an independent predictor of for major adverse CV events, on multivariate analysis.
18752579 data firmly establish the key role of PKC-delta in VEGF-induced VWF release, but suggest that a third, VEGF-specific, signaling intermediate is required as a PKC-delta coactivator.
18725999 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
18724972 Observational study of gene-disease association. (HuGE Navigator)
18712522 Loss of heterozygosity at the cDNA level suggests a lack of expression of the p.H895 allele and a severe von Willebrand phenotype.
18690339 C570S mutation in the VWF propeptide abolishes multimerization of VWF
18665926 delExon1-3 and 2435delC make up 37.5% of the genetic defects in Hungarian patients with VWD type 3.
18647229 The disulfide bonds between GPIbalpha and GPIbbeta are necessary for optimal GPIbalpha binding to VWF
18637125 the N1421K substitution in the VWF affects the GPIb binding site or a recognition element by a conformational change of the A1 domain.
18618671 Observational study of gene-disease association. (HuGE Navigator)
18612539 Report effects of von Willebrand factor concentration and platelet collision on shear-induced platelet activation
18600088 the alpha 2A adrenergic receptor 2372A/G polymorphism is associated with plasma von Willebrand factor levels in a general population.
18600088 Observational study of gene-disease association. (HuGE Navigator)
18577516 Grarnzyme B processes ultra-large VWF multimers released from activated endothelial cells under physiological shear.
18549909 VWF may reduce the immunogenicity of FVIII by inhibiting the uptake of FVIII by antigen presenting cells
18535282 Greater plasma fibrinogen and, to some degree, von Willebrand factor were associated with increased rates of hemorrhagic stroke in these prospective studies, whereas Factor VIII was related to it in younger ARIC study participants only
18521510 Data reveal a more critical role for membrane cholesterol in collagen type III-induced than in VWF-induced Ca(2+) signalling.
18521502 Distribution of von Willebrand factor levels in young women with and without bleeding symptoms: influence of ABO blood group and promoter haplotypes.
18521502 Observational study of gene-disease association. (HuGE Navigator)
18515885 there are associations between several AQP2 SNPs and the risk of venous thrombosis, and weak associations with arterial blood pressure, but not with plasma levels of VWF propeptide, VWF or FVIII
18503632 review factors that determine plasma VWF multimer length and filament formation
18492805 FVIII plays a role in regulating proteolytic processing of von Willebrand factor (VWF) by ADAMTS13 under shear stress, which depends on the high-affinity interaction between FVIII and its carrier protein, VWF.
18485092 Shear stress enhances VWF promoter activity and a polymorphic GT repeat element mediates the stress-induced transactivation in endothelial cells
18449422 Three novel candidate VWF mutations with an increased clearance phenotype were identified in type 1 von Willebrand disease.
18433458 certain inflammatory cytokines selectively inhibit ADAMTS13 synthesis without triggering release of its known substrate, vWF.
18433456 the thiol-disulfide state of ultra-large (UL) VWF multimers regulates the adhesion properties of strings on endothelial cells
18388497 Persistent factor VIII-dependent factor X activation on endothelial cells is independent of von Willebrand factor
18347221 VWF is not only a marker but also actually an important effector in the pathogenesis of myocardial infarction. Review.
18344423 most VWF secreted by nonstimulated cells was not constitutive secretion but basal release of a post-Golgi storage organelle, presumably the WPB
18327408 MgSO(4) significantly enhanced the cleavage of the newly released ultra-large von Willebrand factor strings by ADAMTS-13.
18315556 requirement for multimer analysis compared with a mere ratio of VWF functional parameters and VWF:Ag for classification of VWD; even slightly aberrant multimers are highly predictive for the presence of VWF mutations.
18315546 found the prevalence of VWD2B Malmo/New York in the Italian VWD2B population (35 families) to be approximately 9% (three families); the mutation P1266Q is responsible, similarly to P1266L, for the VWD2B Malmo/New York phenotype
18263586 a new mechanism of anthrax coagulopathy affecting the levels and functional activities of both VWF and its natural regulator ADAMTS13. This mechanism may contribute to hemorrhage and thrombosis typical in anthrax.
18194418 VWF levels correlated with age, fibrinogen and C-reactive protein. VWF correlated positively with risk of myocardial infarction.
18173757 Observational study of gene-disease association. (HuGE Navigator)
18094571 Two novel mutations, C858W and T1477I associated with von Willebrand disease were found and expected to contribute to the elucidation of its pathophysiology.
18064343 Von Willebrand factor is involved in Streptococcus sanguis-induced platelet aggregation.
18045606 The ITGB3 GCC-Pro33 haplotype was associated with increased vWF activity.
18042361 In hypertriglyceridemia VWF elevation is a result of endothelial activation and subsequent mobilization of VWF from the arterial wall.
17996280 Endothelial dysfunction exists in atrial fibrillation and is related to dysfuncion of VWF levels.
17975018 N-linked glycans of VWF have a modulatory effect on the interaction with ADAMTS13
17958741 FVIII trafficking to WPBs is independent of Tyr1680 and high-affinity binding to VWF; the structural requirements that determine intracellular co-trafficking differ from those that determine complex assembly in circulation.
17949477 C1584 and blood group O are associated with increased VWF clearance, and clearance contributes to differing VWF level within a given blood group.
17922807 correlation in all groups between the metabolic equivalents (METS) achieved and the degree of change of VWF and FVIII:C levels; significant differences in the effects of exercise on individuals with type 1 and type 2B VWD
17908278 No association between plasma vWF levels in primary graft dysfunction following lung transplantation.
17901248 ADAMTS13 preferentially acts on platelet-VWF complexes under fluid shear stress
17890957 During the second and third gestation trimesters a significant increase (P < 0.05) in FVIII and VWF:Ag levels and a decrease in FPS levels were seen compared with the first trimester.
17883593 The sorting efficiency of IL-8 into the regulated secretory pathway of the endothelium is tightly controlled by the entry of VWF into Weibel-Palade bodies.
17849054 TLR-4 gene polymorphisms are associated with lower levels of P-selectin and von Willebrand factor among hypercholesterolemic patients.
17786534 Examine correlation between von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and factor VIII activity in plasma.
17764538 VWF parameters are reciprocally correlated with ADAMTS-13 activity in severe sepsis and septic shock but have no prognostic value regarding outcome
17723135 studies on human umbilical vein endothelial cells showed co-localization of VWF and OPG in Weibel-Palade bodies. When secretion was induced, OPG remained associated with VWF in extracellular patches of release under biochemical conditions found in blood
17721608 A time-course study revealed that tumor-derived supernatant induces a rapid endothelial secretion of VWF within minutes, inducing platelet adhesion
17681836 study found three novel mutations in Mexican Mestizo patients with von Willebrand disease: E1447Q in one patient with type 1; P2781S in one patient with type 2M; and P812L in another type 1/2N patient
17650077 VWF and fibrinogen are differentially packaged in human platelets
17622488 Investigated microalbuminuria, von Willebrand factor and fibrinogen levels as markers of the severity in chronic obstructive pulmonary disease exacerbation.
17619827 Examine platelet GPIbalpha, GPIV and vWF polymorphisms and fatal pre-hospital myocardial infarction among middle-aged men.
17619827 Observational study of gene-disease association. (HuGE Navigator)
17598021 identified a r1306 nutation in six patients from two unrelated families with subtype 2B vWD
17598011 Observational study of gene-disease association. (HuGE Navigator)
17598011 VWF A/T1381 polymorphism plays an important role in inter-individual variability of the affinity of VWF for GPIbalpha, with T/T variants having a higher affinity than A/A and A/T variants
17597991 review of the role of von Willebrand factor and its primary platelet receptor, glycoprotein Ib, in the complex responses to vascular injury
17493665 A novel mechanism has been demonstrated for initiating thrombus formation under high haemodynamic forces that involves alpha(IIb)beta(3)-independent platelet aggregation at the interface between immobilised and soluble VWF
17490730 the molecular correlates for type 1 VWD are complex and, in addition to a wide array of changes at the VWF locus, are likely to involve mutations in other genes
17472573 It was concluded that, via inhibition of protein geranylgeranylation, fluvastatin is a broadspectrum inhibitor of regulated von Willebrand factor secretion.
17414220 PURPOSE OF REVIEW: 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.
17408410 both the gpIb-VWF interaction and the integrin alpha(2)beta(1)-collagen interaction contribute to platelet adhesion under high shear stress; integrin alpha(II)beta(1) makes a greater contribution to adhesion to type I collagen because less VWF is bound
17408405 analysis of how VWF antigen (VWF:Ag) and ristocetin cofactor activity (VWF:RCo) contribute to development of type 1 von Willebrand disease
17393013 Proper conformation of the B2 domain, which depends on critical Cys residues, may be required for the normal proteolytic processing of vWF multimers.
17393012 Soluble VWF multimers isolated from human plasma self-assemble to a network of fibers immobilized on a collagen matrix and are functionally active to bind platelets.
17371490 VWF and TMEM16B deletions may have a role in severe von Willebrand disease type 3
17296575 the L1565 variant of von Willebrand factor has a role in susceptibility to proteolysis by ADAMTS13
17275499 The endothelial reperfusion injury after liver transplantation shows an increase in von Willebrand factor.
17190853 Study of 123 type 1 von Willebrand disease patients found 50 different mutations: 31 missense, 8 in the VWF transcriptional regulatory region, 5 deletions/insertions, 5 splicing consensus sequence ones & 1 nonsense. 15 new sequence variations were found.
17159339 complexity of nucleotide sequence structure of intron 40 region shown by three polymorphic tetranucleotide repeat subloci SSR 'a', SSR 'b' and SSR 'c' within the same originally described locus
17159336 role of vWF and its relationship to platelet activation in the presence of central venous lines in sick neonates
17146059 The specificity of this unique mechanism depends on tension-induced unfolding of the VWF A2 domain, which exposes the scissile bond and exosite for interaction with complementary sites on ADAMTS13
17137217 Observational study of gene-disease association. (HuGE Navigator)
17121983 Ionic interactions of the Pro-1645-Lys-1668 region of the von Willebrand factor A2 domain with exosite on ADAMTS-13 play a significant role in mediating substrate recognition.
17119126 Observational study of gene-disease association and gene-gene interaction. (HuGE Navigator)
17119126 a qualitative difference in Y/C1584 plasma VWF multimer profile was observed compared with that for Y/Y1584 VWF
17109387 A novel null mutation (2908del C in exon 22) of the VWF gene was found in a compound heterozygote bearing the C2362F mutation, which causes bleeding only in the compound heterozygous or homozygous state.
17100655 VWF activates endothelial nitric oxide synthase through a specific Ca2+-dependent Glycoprotein Ib receptor-signaling cascade that relies on the generation of platelet-derived adenosine diphosphate and thromboxane A2.
17098004 Patients wiwth cardiac allograft vasculopathy showed significantly higher vWf serum concentrations.
17090649 O-linked glycosylation profile of VWF stored in endothelial storage organelles may differ from circulating VWF
17087728 The susceptibility of VWF mutants to proteolysis of ADAMTS-13 decreased in the following order: von Willebrand disease type 2A > type 2B > type 2M > rVWF-WT
17053057 Similar to VWF, the estimated risk of myocardial infarction was increased for every quartile of ADAMTS13 when compared to the lowest quartile
17052980 multimeric VWF appears to be a loosely packed ellipsoidal protein with non-covalent interactions between different monomer units stabilizing its solution structure
17010412 Glycoprotein Ib (GPIb) binding domain in vWF undergoes changes when adsorbed on collagen VI compared to vWF on a hydrophobic synthetic surface.
16990571 von Willebrand factor antigen and activity are associated with the occurrence of acute ischemic stroke.
16985174 105 of 150 type 1 von Willebrand disease patients had mutations identified. A subgroup with abnormal multimers (57 of 150) had a high prevalence of VWF gene mutations (54 of 57). Those with qualitatively normal VWF had fewer mutation(51 of 93).
16953272 neither FVIII, thrombin-activated FVIII, VWF nor a complex of FVIII and VWF modulate the maturation of human dendritic cells or their capacity to stimulate autologous or allogeneic T cells
16953269 R763G is a new type 2N VWD mutation located in the VWF propeptide which alters the proteolytic processing of VWF and consequently its binding to FVIII
16899464 the A1 domain of VWF contains the binding site of chloride ions that control allosterically the proteolysis by ADAMTS-13 of the Tyr1605-Met1606 bond in the A2 domain
16894469 analysis of the relationship between a mutation in exon 21 of the von Willebrand factor gene and the mild bleeding phenotype
16870550 The P1824H substitution in the A3 domain is associated with very low VWF levels and with a moderate-to-severe bleeding tendency to Von Willebrand's disease.
16839358 Dopamine, D2/D3- and D4-specific agonists inhibited histamine- but not thrombin-induced VWF secretion; the dopamine effects are not mediated by [Ca(2+)](i)-dependent signalling or cAMP-mediated signaling
16835381 the ratio of steady-state plasma VWFpp to VWF:Ag can be used to identify patients with a shortened VWF half-life; DNA sequencing revealed a new single (heterozygous) VWF mutation in affected individuals, S2179F and W1144G.
16805860 in shear forces the VWF A1 domain can assume the role of A3 to trigger substantial platelet recruitment to human collagen fibres
16774914 Data demonstrate that LRR2-4, encompassing a pronounced negative charge patch on human glycoprotein (GP)Ibalpha, is essential for GPIbalpha.von Willebrand factor-dependent adhesion as hydrodynamic shear increases.
16735600 fibronectin assembly has a role in platelet thrombus formation in response to type I collagen and von Willebrand factor
16676067 mutation A2801D disturbs the folding of the CK domain, which may result in a mixture of monomers and dimers of V
16634765 ADAMTS-13 activity, von Willebrand factor level and platelet activation are modified by physical stress (letter)
16449527 a biomechanical process mediated by GPIbalpha-VWF bonds in rapidly flowing blood may not only initiate platelet arrest onto reactive vascular surfaces but also generate procoagulant microparticles that further enhance thrombus formation
16435187 analysis of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia
16424652 Observational study of genetic testing. (HuGE Navigator)
16420575 direct binding to collagen induces a change in the structural conformation within the VWF-A1 domain, and the G1324S substitution prevents this conformational change
16418330 lumenal domain of P-selectin interacts with the D'-D3 domains of VWF in Weibel-Palade bodies.
16373331 domain translocation in VWF allows interaction with GPIbalpha
16324758 A marker of endothelial damage/injury in the activation of vascular and coagulation abnormalities in acute coronary syndromes.
16322474 results imply that increased VWF susceptibility for ADAMTS13 is a constitutive property of classical VWD type 2A in an investigation of the impact of mutations commonly found in patients with VWD type 2A on ADAMTS13-dependent proteolysis of VWF
16321553 Thirty-two novel sequence variations were identified (2 deletions, 2 nonsense, 15 missense, 6 silent, and 7 intronic), some with clear functional consequences.
16320153 Observational study of gene-disease association. (HuGE Navigator)
16320153 the Thr789Ala vWF gene variant is associated with increased plasma concentrations of vWF, ristocetin co factor levels and collagen binding capacity in patients with type 2 diabetes and coronary heart disease
16314412 von Willebrand factor A3-domain binds to the antithrombotic antibody 82D6A3, as shown by X-ray crystallography
16286459 substrate recognition of ADAMTS13 for VWF depends on structural features or exosites on multimeric VWF that are missing from FRETS-VWF73
16247740 genetic analysis showed that the mutation R1374C is responsible for type 2M VWD. A high frequency of the R1374C mutation is observed in northwestern Spain
16221672 ADAMTS13 controls the multimeric size of circulating von Willebrand factor by reacting at several binding sites.
16203734 truncation of the ADAMTS13 protein at its cysteine-rich region eliminates its recognition by the antibodies without abolishing its von Willebrand factor-cleaving activity
16194200 The effect of three cysteine-mutations (C1130F, C1149R or C2671Y) on the in vivo survival of VWF was studied in patients carrying these mutations and in a VWF-deficient mice model
16141351 CUB-1 domain may serve as the docking site for ADAMTS13 to bind unusually large forms of von Willebrand factor (ULVWF) under flow, a critical step to initiate ULVWF proteolysis
16102037 ultra-large von Willebrand factor is cleaved by ADAMTS-13 under flow conditions (review)
16102036 von Willebrand factor has roles in minor bleeding and clotting as well as in hemorrhage or thrombosis [review]
15975930 the proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for recognition and cleavage of von Willebrand factor between amino acid residues Asp(1595) and Arg(1668).
15946221 Endothelial microparticles carry unusually large vWf multimers
15946218 reveal a V980-P981-W982 sequence that contributes to alpha(IIb)beta3 activation in in vitro; VPW accelerated the activation of the tyrosine kinase Syk which controls cytoskeletal rearrangements
15933060 a gain-of-function phenotype resulting from mutations in the LRR region of GP Ibalpha; data suggest that the LRRs regulate GP Ibalpha affinity for VWF allosterically
15886817 Observational study of genetic testing. (HuGE Navigator)
15886321 reduction in the number of terminal sugars on N-linked glycan increases susceptibility of globular VWF to ADAMTS13 proteolysis and is associated with reduced plasma VWF:Ag and VWF:CB levels.
15849757 Elevated levels of plasma vWF anatigen are related to Behcet disease exacerbation rather than vascular involvement.
15842375 the VWF Y1584C polymorphism may be associated with bleeding in vitamin K antagonist-treated patients [letter]
15842374 analysis of folding of mutant VWF proteins that may have roles in Group I and Group II, type 2A von Willebrand disease
15842373 there is a possible association between VWF levels and arterial rigidity, but not intima-media thickness [letter]
15831502 analysis of kinetics of cell surface loss of von Willebrand factor and its propolypeptide after secretion from Weibel-Palade bodies in living human endothelial cells
15824096 analysis of ADAMTS13 binding to von Willebrand factor
15816602 The vWf antigen levels of non-small cell lung cancer patients are not substantially altered from those of normal controls
15809291 the interaction between von Willebrand factor and ADAMTS-13 is modulated by chloride ions
15799029 Intracellular localization of OPG in HUVEC, in association with vWF, together with its rapid and sustained secretory response to inflammatory stimuli, strongly support a modulatory role in vascular injury, inflammation and hemostasis.
15764659 importance of the bond kinetics associated with the VWF/GP Ib-IX interaction in hemostasis, and the drastic changes in binding that can occur when only a single amino acid of GP Ibalpha is altered
15748247 leukocytes tethered to and rolled on platelet-decorated ultra-large VWF strings, but not directly on endothelial cells, under high shear stresses of 20 and 40 dyn/cm(2) in a P-selectin dependent manner
15748246 specific VWF self-association under static conditions is demonstrated; results suggest a multiple domain interaction between immobilized and soluble VWF
15735796 Observational study of gene-disease association. (HuGE Navigator)
15514009 the interaction of platelet GP Ib with VWF mediates the activation of alpha2beta1, increasing its affinity for collagen
15471879 hemophilic mutations across the factor VIII C2 domain have variable effects on stability and von Willebrand Factor-binding activities
15467896 higher amounts of neonatal vWf (compared to adult VWF) attached to platelets in the presence of shear stress or ristocetin; this efficient neonatal vWf-platelet interaction is an effect intrinsic to the neonatal vWf, and not to the neonatal platelet
15459008 VWF stimulates the platelet adhesion onto the collagen surface by enhancing platelet aggregation in the platelet-reduced condition.
15382239 strong indications for the existence of an exposed tryptophan rotamer conformation
15377475 A G1629R mutation was identified in an Italian patient with type 2A von Willebrand disease. It contained intermediate & HMW multimers. Binding of R1629-rVWF to platelet GPIb was moderately reduced.
15331450 Expression of mutant VWF proteins confirmed that VWF multimerization is not necessary for Weibel-Palade body creation
15322948 Molecular dynamics simulation was used to study the structural and dynamic effects of eight mutations introduced into the von Willebrand factor A2 Domain.
15257287 siRNA-mediated downregulation of annexin A2/S100A10 and disruption of the complex by microinjection of peptide competitors result in a marked reduction in vWF
15249683 Results suggest that von Willebrand factor domain A1 inhibits the cleavage of domain A2 by ADAMTS13, and that inhibition can be relieved by interaction of domain A1 with platelet platelet glycoprotein Ibalpha or certain glycosaminoglycans.
15226181 base substitution located at the last nucleotide of exon 26 produces a transcript with the in-frame deletion of exon 26, and a transcript with the in-frame deletions of exons 23 and 26, which led to intracellular retention of VWF
15219197 Observational study of genotype prevalence. (HuGE Navigator)
15219197 the VWF gene from a Type 2A VWD patient showed a novel, heterozygous T-->A transversion at nucleotide 4510, resulting in the L1503Q usbstitution; an aberrant VWF multimer pattern was observed; the protein was digested with the ADAMTS13 protease
15203711 showed role for vWF that would synergise with collagen leading to functional activation and showed this to be the case for platelet aggregation, 5-HT secretion and calcium responses.
15182579 C1423T polymorphism of VWF is low frequency in both controls and patients of Han population.
15150074 An endothelial-specific protein DNA complex was formed with an oligonucleotide that corresponded to sequences +155 to +184 of the VWF gene
15102023 results suggest that much of the difference in prothrombotic potency of different collagens is directly related to their differences in VWF content; found that fibrillar collagen, but not acid-soluble collagen, contains a substantial quantity of VWF
15087444 Willebrand factor-dependent shear-induced platelet aggregation is augmented through Ca2+-calmodulin and myosin light chain kinase activation
15079089 determination of domain in capillary morphogenesis protein 2
15005754 Observational study of gene-disease association. (HuGE Navigator)
14995987 how missense mutations resulting in the loss of cysteine residues in the D3-domain (multimerization area) and in the carboxy-terminus (dimerization area) result in a VWF deficiency and how they interfere with dimerization and multimerization processes.
14755371 Case report of a missense mutation found in type 2A von Willebrand disease which disrupts interaction with GP Ib-IX.
14727255 REVIEW: processing of von Willebrand factor by ADAMTS-13 on endothelial cells
14727254 REVIEW: degradation in thrombotic thrombocytopenic purpura
14717981 polymerizing fibrin interacts with VWF so as to activate GPIb
14717980 pathological shear-induced VWF binding to GpIb-IX-V stimulates PIP3 production through the assembly of an alpha-actinin-based complex that colocalizes PI 3-KIA with substrate PIP2
14717782 Observational study of gene-disease association. (HuGE Navigator)
14691575 vWF-induced platelet activation involves p38 MAP kinase
14630802 P-selectin may anchor ultralarge VWF strings to endothelial cells and facilitate their cleavage by ADAMTS13.
14604963 fibrin-bound VWF has two roles in thrombus formation: recruiting platelets by way of glycoprotein (GP) Ib, and contributing to stationary platelet adhesion by way of binding to activated alphaIIbbeta3
14525793 increased susceptibility to proteolysis tracked with the "G" allele of the A/G polymorphism at position 24/1282, encoding the amino acid polymorphism Tyr/Cys1584 ("G" = Cys1584)
14517489 studied a group of healthy non-bleeding women to evaluate normal ranges OF F8 and VWF and their relationship to blood group and parity in normal pregnancy, and the return to non-pregnant factor levels in puerperium
14512308 VWF73 is a specific substrate for ADAMTS-13
14507115 data suggest a common mechanism for platelet and vWF abnormalities in these chronically hypoxic pulmonary hypertension patients
12958606 antibodies against FVIII acidic regions can inhibit FVIII function by a variety of mechanisms, in particular by interfering with the binding of FVIII to phospholipids & VWF.
12883448 Data show that Von Willebrand factor was significantly increased in diabetic patients compared to controls, and was significantly higher in patients with micro/macroalbuminuria than those with normoalbuminuria.
12871466 platelet adhesion to fibrin at a shear rate of 1000 s(-1) requires fibrin-bound VWF
12860973 To determine the sequence in the beta3 cytoplasmic domain that is critical to integrin signaling, cell lines were established that coexpress VWD.
12851529 A fragment of approximately 60 kDa that co-purified with FN, with affinity to heparin and gelatin, has the activity that controls vWF multimer size.
12815111 Data show that platelet activation correlates with binding of von Willebrand factor to platelets.
12792699 Observational study of gene-disease association. (HuGE Navigator)
12791651 ability of VWF to drive both the formation of a storage compartment and the function of that compartment with respect to regulated secretion.
12775718 ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor
12737944 In a group of 40 VWD type 3 patients (Italians, Iranians, and Indians), 50 gene defects were identified, of which 45 are novel; the mutations are scattered throughout the entire VWF gene,revealing no founder effect in these three populations studied.
12729795 These observations indicate that von Willebrand factor plays an active role in sequestering interleukin-8 into Weibel-Palade bodies.
12719775 Competitive ELISAs suggested that FACTOR VIII F2200 plays a more important role in both phospholipid-binding and vWF-binding than N2198 and M2199.
12676191 The interaction of VWF with GP Ibalpha mediates the translocation of CD40L to the platlet cell surface when the platelets are exposed to a high shear stress
12668663 Blood platelets are exposed to a purified VWF substrate, providing a relatively high concentration of binding sites for the platelet glycoprotein CGPIIb-IIIa complex.
12649144 A type 1 VWF disease gene was sequenced, revealing 2 sequence variations: intron 30 (5312-19A>C) and exon 28 at Tyr1584Cys (4751A>G). No significant structural changes occur from this substitution but a new solvent-exposed reactive thiol group is seen.
12646253 data demonstrate residues D1323(560) and G1324(561) being critical for vWF-A1 mediated platelet aggregation
12624629 the strongest linkage signal (LOD =3.46, p = 0.00003) for vWF was found on chromosome 9q34 at the DNA marker D9S290, where the ABO gene is located
12582178 Surface characteristics of the collagen-binding site of the A3 domain were ellicited using 22 point mutations as a mapping tool; docking studies suggested possible engagements of a collagen triple helix interacting with A3
12579041 REVEIW: structural and functional properties of VWF domains; their interaction with glycoprotein Ibalpha, and their role in platelet activation and thrombus formation
12543870 Enhanced interaction of 2B-rVWF with GPIb, without engagement of alphaIIbbeta3, is sufficient to induce shear-induced platelet aggregation, but does not lead to stable thrombus formation.
12511565 inhibition of promoter activation by nuclear factor Y transcription factor
12456504 studies demonstrating that fluid shear stress rather than shear rate controls platelet activation show for the first time the ability of hydrodynamic forces to induce VWF aggregation in suspension, that may control cell adhesion rates in circulation
12447349 The binding site of the A3 domain of von Willebrand factor was located at its hydrophobic 'front' surface.
12445472 Calcium-regulated secretion of von Willebrand factor in vascular endothelial cells.
12393736 Interaction between von Willebrand factor and glycoprotein Ib activates Src kinase in human platelets.
12393698 gentic variant demonstrates that a normal consensus sequence for VWF propeptide cleavage and efficient cleavage are required in vivo for normal FVIII binding capacity of VWF
12393671 impaired thrombus generation in type 2B VWD ascribed to the critical function of larger VWF multimers in the proper spatial growth of mural thrombi under high shear rate conditions.
12381897 Observational study of gene-disease association. (HuGE Navigator)
12381897 Polymorphisms in the von Willebrand factor gene are not associated with proliferative retinopathy in non-insulin-dependent diabetes mellitus. Two polymorphisms (-1793G/C and Thr789Ala) in the von Willebrand factor gene with PDR.
12362242 The mucin-like macroglycopeptide region of glycoprotein Ibalpha is required for cell adhesion to immobilized von Willebrand factor (VWF) under flow but not for static VWF binding.
12361948 PKA-mediated phosphorylation of GPIbbeta at Ser(166) negatively regulates VWF binding to GPIb-IX and is one of the mechanisms by which PKA mediates platelet inhibition
12215337 Expression in uterine leiomyoma
12183630 structures of the glycoprotein Ibalpha amino-terminal domain and its complex with the VWF domain A1 are presented
12163004 REVIEW: Control of VWF multimer size by VWF-cleaving protease and thrombospondin-1 and role in arterial thrombosis.
12091053 Observational study of genotype prevalence. (HuGE Navigator)
12083486 Factor VIIa induces release of von Willebrand factor from human umbilical vein endothelial cells by a tyrosine kinase dependent pathway.
12082590 Observational study of gene-disease association. (HuGE Navigator)
12070018 the rGPIa/IIa-collagen interaction dominates the adhesion of rGPIa/IIa-Ib alpha-liposomes to the collagen surface at low shear rates; the rGPIa/IIa-collagen and rGPIb alpha-VWF interactions synergistically support liposome adhesion at high shear rates.
12069583 Binding of VFW to the modulator, snake venom protein bitiscetin, has been defined at a distinct site in the A1 domain of VWF spanning over alpha4a, alpha5 helices and the loop between alpha5 and beta6 close to the botrocetin- and mAb NMC-4-binding sites.
12036879 Endothelial VWF recruits platelets to atherosclerosis-prone sites in response to hypercholesterolemia. Hypercholesterolemia primes platelets for recruitment via VWF, GPIb alpha, and P-selectin before lesions are detectable.
12036871 Site-directed mutagenesis of platelet glycoprotein Ib alpha demonstrates residues crucial for botrocetin-mediated VWF binding.
12006654 the ability of VWF pseudogranules, which are secretagogue responsive, to recruit membrane proteins
11992244 role in the preservation of factor VIII in the circulation is investigated by administration of human VWF and pro-VWF in murine and dog models of vWD.
11992236 REVIEW: current knowledge on the role of vWF in primary hemostasis is reviewed.
11943773 Identification of the regulatory elements of the human von Willebrand factor for binding to platelet GPIb
11920243 Arg 1715 is not essential in the function but it is necessary for maintaining the conformation recognized by MoAb 9 specific for the GPIIb/IIIa-binding domain of VWF.
11914659 raised vWf in breast cancer seems likely to be independent of the acute phase response
11877281 Shear-dependent morphology of von Willebrand factor bound to immobilized collagen
11869299 Elevation of vWF in plasma was associated with neuropathic foot ulceration, linking endothelial dysfunction to foot ulceration
11864703 vWF and tPAag but not thrombomodulin in the present population are independent markers of atherosclerosis.
11859851 In several genetic subtypes of VWF disease, the F8/VWF ratio increases as VWF synthesis is reduced. It remains 1 when VWF clearance increases. A high F8/VWF ratio & a low VWF level may indicate a true genetic defect, possibly a null allele.
11843298 Mutations in particular domains of VWF correlate with specific subtypes of type 2 von Willebrand disease depending on how they affect its post-translational processing, multimerization, secretion, or binding.
11843287 VWF has roles in hemostasis, platelet adhesion, and inflammation.
11843285 VWF with a unique multimeric structure plays a pivotal role in hemostasis and pathological intravascular thrombosis by contributing to platelet function and blood coagulation through its multiple adhesive functions for platelet membrane receptors.
11776314 4 mutations, at C509, V551, R552 and R611 lead to decreased binding to heparin in plasma and rVWF. No heparin-binding defect was found with different type 2 VWF mutants, at positions G561, E596, I662, R543, R545, V553, R578 or L697.
11776313 Incubation of a recombinant provWF (rpvWF) preparation with canine and human vWF-deficient plasma induced a time-dependent decrease in provWF antigen and an increase in vWFpp antigen without changing total vWF antigen or collagen-binding activity.
11756664 Our findings highlight a newly recognized role of circulating VWF in the initiation of platelet adhesion
11755948 Observational study of gene-disease association. (HuGE Navigator)
11736957 Observational study of gene-disease association. (HuGE Navigator)
11722428 Observational study of gene-disease association. (HuGE Navigator)
11583318 Ser968Thr mutation in the A3 domain leads to defective binding to collagen and bleeding tendency.
11236861 Observational study of genotype prevalence. (HuGE Navigator)

AA Sequence

MIPARFAGVLLALALILPGTLCAEGTRGRSSTARCSLFGSDFVNTFDGSMYSFAGYCSYLLAGGCQKRSF      1 - 70
SIIGDFQNGKRVSLSVYLGEFFDIHLFVNGTVTQGDQRVSMPYASKGLYLETEAGYYKLSGEAYGFVARI     71 - 140
DGSGNFQVLLSDRYFNKTCGLCGNFNIFAEDDFMTQEGTLTSDPYDFANSWALSSGEQWCERASPPSSSC    141 - 210
NISSGEMQKGLWEQCQLLKSTSVFARCHPLVDPEPFVALCEKTLCECAGGLECACPALLEYARTCAQEGM    211 - 280
VLYGWTDHSACSPVCPAGMEYRQCVSPCARTCQSLHINEMCQERCVDGCSCPEGQLLDEGLCVESTECPC    281 - 350
VHSGKRYPPGTSLSRDCNTCICRNSQWICSNEECPGECLVTGQSHFKSFDNRYFTFSGICQYLLARDCQD    351 - 420
HSFSIVIETVQCADDRDAVCTRSVTVRLPGLHNSLVKLKHGAGVAMDGQDVQLPLLKGDLRIQHTVTASV    421 - 490
RLSYGEDLQMDWDGRGRLLVKLSPVYAGKTCGLCGNYNGNQGDDFLTPSGLAEPRVEDFGNAWKLHGDCQ    491 - 560
DLQKQHSDPCALNPRMTRFSEEACAVLTSPTFEACHRAVSPLPYLRNCRYDVCSCSDGRECLCGALASYA    561 - 630
AACAGRGVRVAWREPGRCELNCPKGQVYLQCGTPCNLTCRSLSYPDEECNEACLEGCFCPPGLYMDERGD    631 - 700
CVPKAQCPCYYDGEIFQPEDIFSDHHTMCYCEDGFMHCTMSGVPGSLLPDAVLSSPLSHRSKRSLSCRPP    701 - 770
MVKLVCPADNLRAEGLECTKTCQNYDLECMSMGCVSGCLCPPGMVRHENRCVALERCPCFHQGKEYAPGE    771 - 840
TVKIGCNTCVCQDRKWNCTDHVCDATCSTIGMAHYLTFDGLKYLFPGECQYVLVQDYCGSNPGTFRILVG    841 - 910
NKGCSHPSVKCKKRVTILVEGGEIELFDGEVNVKRPMKDETHFEVVESGRYIILLLGKALSVVWDRHLSI    911 - 980
SVVLKQTYQEKVCGLCGNFDGIQNNDLTSSNLQVEEDPVDFGNSWKVSSQCADTRKVPLDSSPATCHNNI    981 - 1050
MKQTMVDSSCRILTSDVFQDCNKLVDPEPYLDVCIYDTCSCESIGDCACFCDTIAAYAHVCAQHGKVVTW   1051 - 1120
RTATLCPQSCEERNLRENGYECEWRYNSCAPACQVTCQHPEPLACPVQCVEGCHAHCPPGKILDELLQTC   1121 - 1190
VDPEDCPVCEVAGRRFASGKKVTLNPSDPEHCQICHCDVVNLTCEACQEPGGLVVPPTDAPVSPTTLYVE   1191 - 1260
DISEPPLHDFYCSRLLDLVFLLDGSSRLSEAEFEVLKAFVVDMMERLRISQKWVRVAVVEYHDGSHAYIG   1261 - 1330
LKDRKRPSELRRIASQVKYAGSQVASTSEVLKYTLFQIFSKIDRPEASRITLLLMASQEPQRMSRNFVRY   1331 - 1400
VQGLKKKKVIVIPVGIGPHANLKQIRLIEKQAPENKAFVLSSVDELEQQRDEIVSYLCDLAPEAPPPTLP   1401 - 1470
PDMAQVTVGPGLLGVSTLGPKRNSMVLDVAFVLEGSDKIGEADFNRSKEFMEEVIQRMDVGQDSIHVTVL   1471 - 1540
QYSYMVTVEYPFSEAQSKGDILQRVREIRYQGGNRTNTGLALRYLSDHSFLVSQGDREQAPNLVYMVTGN   1541 - 1610
PASDEIKRLPGDIQVVPIGVGPNANVQELERIGWPNAPILIQDFETLPREAPDLVLQRCCSGEGLQIPTL   1611 - 1680
SPAPDCSQPLDVILLLDGSSSFPASYFDEMKSFAKAFISKANIGPRLTQVSVLQYGSITTIDVPWNVVPE   1681 - 1750
KAHLLSLVDVMQREGGPSQIGDALGFAVRYLTSEMHGARPGASKAVVILVTDVSVDSVDAAADAARSNRV   1751 - 1820
TVFPIGIGDRYDAAQLRILAGPAGDSNVVKLQRIEDLPTMVTLGNSFLHKLCSGFVRICMDEDGNEKRPG   1821 - 1890
DVWTLPDQCHTVTCQPDGQTLLKSHRVNCDRGLRPSCPNSQSPVKVEETCGCRWTCPCVCTGSSTRHIVT   1891 - 1960
FDGQNFKLTGSCSYVLFQNKEQDLEVILHNGACSPGARQGCMKSIEVKHSALSVELHSDMEVTVNGRLVS   1961 - 2030
VPYVGGNMEVNVYGAIMHEVRFNHLGHIFTFTPQNNEFQLQLSPKTFASKTYGLCGICDENGANDFMLRD   2031 - 2100
GTVTTDWKTLVQEWTVQRPGQTCQPILEEQCLVPDSSHCQVLLLPLFAECHKVLAPATFYAICQQDSCHQ   2101 - 2170
EQVCEVIASYAHLCRTNGVCVDWRTPDFCAMSCPPSLVYNHCEHGCPRHCDGNVSSCGDHPSEGCFCPPD   2171 - 2240
KVMLEGSCVPEEACTQCIGEDGVQHQFLEAWVPDHQPCQICTCLSGRKVNCTTQPCPTAKAPTCGLCEVA   2241 - 2310
RLRQNADQCCPEYECVCDPVSCDLPPVPHCERGLQPTLTNPGECRPNFTCACRKEECKRVSPPSCPPHRL   2311 - 2380
PTLRKTQCCDEYECACNCVNSTVSCPLGYLASTATNDCGCTTTTCLPDKVCVHRSTIYPVGQFWEEGCDV   2381 - 2450
CTCTDMEDAVMGLRVAQCSQKPCEDSCRSGFTYVLHEGECCGRCLPSACEVVTGSPRGDSQSSWKSVGSQ   2451 - 2520
WASPENPCLINECVRVKEEVFIQQRNVSCPQLEVPVCPSGFQLSCKTSACCPSCRCERMEACMLNGTVIG   2521 - 2590
PGKTVMIDVCTTCRCMVQVGVISGFKLECRKTTCNPCPLGYKEENNTGECCGRCLPTACTIQLRGGQIMT   2591 - 2660
LKRDETLQDGCDTHFCKVNERGEYFWEKRVTGCPPFDEHKCLAEGGKIMKIPGTCCDTCEEPECNDITAR   2661 - 2730
LQYVKVGSCKSEVEVDIHYCQGKCASKAMYSIDINDVQDQCSCCSPTRTEPMQVALHCTNGSVVYHEVLN   2731 - 2800
AMECKCSPRKCSK                                                            2801 - 2813
//

Text Mined References (752)

PMID Year Title
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26773038 2016 N-linked glycan stabilization of the VWF A2 domain.
26744078 2016 A role of stochastic phenotype switching in generating mosaic endothelial cell heterogeneity.
26677223 2016 Mutational Constraints on Local Unfolding Inhibit the Rheological Adaptation of von Willebrand Factor.
26670633 2016 von Willebrand factor is dimerized by protein disulfide isomerase.
26645283 2015 Apoptotic Platelet Events Are Not Observed in Severe von Willebrand Disease-Type 2B Mutation p.V1316M.
26616301 2016 Acquired von Willebrand factor deficiency caused by LVAD is ADAMTS-13 and platelet dependent.
26600159 2015 Genome-Wide Association Study Identifies That the ABO Blood Group System Influences Interleukin-10 Levels and the Risk of Clinical Events in Patients with Acute Coronary Syndrome.
26595154 2016 Keeping von Willebrand Factor under Control: Alternatives for ADAMTS13.
26581637 2016 Free thiol groups in von Willebrand factor (VWF) are required for its full function under physiological flow conditions.
26552698 2016 High-density lipoprotein modulates thrombosis by preventing von Willebrand factor self-association and subsequent platelet adhesion.
26473492 2015 Factor VIII Is Synthesized in Human Endothelial Cells, Packaged in Weibel-Palade Bodies and Secreted Bound to ULVWF Strings.
26345337 2015 The co-influence of VWD type 2B/2M mutations in the A1 domain and platelet GPIb? on the rate of cleavage to VWF by ADAMTS13.
26307534 2015 Free hemoglobin increases von Willebrand factor-mediated platelet adhesion in vitro: implications for circulatory devices.
26266817 2015 SNAP23 Regulates Endothelial Exocytosis of von Willebrand Factor.
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26223854 2015 Flow-driven assembly of VWF fibres and webs in in vitro microvessels.
26178390 2015 Pathological von Willebrand factor fibers resist tissue plasminogen activator and ADAMTS13 while promoting the contact pathway and shear-induced platelet activation.
26168189 2015 Von Willebrand Factor, ADAMTS13 and D-Dimer Are Correlated with Different Levels of Nephropathy in Type 1 Diabetes Mellitus.
26121272 2015 High levels of von Willebrand factor and low levels of its cleaving protease, ADAMTS13, are associated with stroke in young HIV-infected patients.
26088471 2015 Mutations in the D1 domain of von Willebrand factor impair their propeptide-dependent multimerization, intracellular trafficking and secretion.
26047609 2015 Combined genetic effects of EGLN1 and VWF modulate thrombotic outcome in hypoxia revealed by Ayurgenomics approach.
26019279 2015 Variable content of von Willebrand factor mutant monomer drives the phenotypic variability in a family with von Willebrand disease.
25983111 2015 The function of ultra-large von Willebrand factor multimers in high shear flow controlled by ADAMTS13.
25973092 2015 Roles of claudin-5 and von Willebrand factor in patients with rheumatoid arthritis.
25916953 2016 Assessment of the Diagnostic Value of Plasma Levels, Activities, and Their Ratios of von Willebrand Factor and ADAMTS13 in Patients with Cerebral Infarction.
25904363 2015 The p.R1819_C1948delinsS mutation makes von Willebrand factor ADAMTS13-resistant and reduces its collagen-binding capacity.
25886574 2015 Gastric cancer-associated enhancement of von Willebrand factor is regulated by vascular endothelial growth factor and related to disease severity.
25876231 2015 Qualitative and quantitative modifications of von Willebrand factor in patients with essential thrombocythemia and controlled platelet count.
25846964 2015 Development of a Hyperactive Primary Hemostatic System During Off-Pump Lung Transplantation Resulting From an Unbalance Between von Willebrand Factor and Its Cleaving Protease ADAMTS13.
25832887 2015 CLEC4M and STXBP5 gene variations contribute to von Willebrand factor level variation in von Willebrand disease.
25810255 2015 Force-induced on-rate switching and modulation by mutations in gain-of-function von Willebrand diseases.
25782102 2015 Unconjugated bilirubin inhibits proteolytic cleavage of von Willebrand factor by ADAMTS13 protease.
25780857 2015 Evaluation of von Willebrand factor phenotypes and genotypes in Hemophilia A patients with and without identified F8 mutations.
25779970 2015 Gene-centric approach identifies new and known loci for FVIII activity and VWF antigen levels in European Americans and African Americans.
25756206 2015 Changes in von Willebrand factor level and von Willebrand activity with age in type 1 von Willebrand disease.
25712991 2015 von Willebrand factor biosynthesis, secretion, and clearance: connecting the far ends.
25673639 2015 von Willebrand factor propeptide and the phenotypic classification of von Willebrand disease.
25670067 2015 Von Willebrand factor as a biological sensor of blood flow to monitor percutaneous aortic valve interventions.
25650553 2015 No evidence for a direct effect of von Willebrand factor's ABH blood group antigens on von Willebrand factor clearance.
25646961 2015 Identification of an interaction between VWF rs7965413 and platelet count as a novel risk marker for metabolic syndrome: an extensive search of candidate polymorphisms in a case-control study.
25612915 2015 Interaction of von Willebrand factor with platelets and the vessel wall.
25608503 2015 Mechanism and functional impact of CD40 ligand-induced von Willebrand factor release from endothelial cells.
25605439 2015 Interaction between VWF and FVIII in treating VWD.
25512528 2014 Allosteric activation of ADAMTS13 by von Willebrand factor.
25498844 2015 Relationship between ADAMTS13 activity, von Willebrand factor antigen levels and platelet function in the early and late phases after TIA or ischaemic stroke.
25472874 2015 Von Willebrand factor in relation to coronary plaque characteristics and cardiovascular outcome. Results of the ATHEROREMO-IVUS study.
25439775 2015 Pathologic von Willebrand factor degradation with a left ventricular assist device occurs via two distinct mechanisms: mechanical demolition and enzymatic cleavage.
25425019 2015 Predictors of von Willebrand disease diagnosis in individuals with borderline von Willebrand factor plasma levels.
25420483 2014 Low paraoxonase 1 arylesterase activity and high von Willebrand factor levels are associated with severe coronary atherosclerosis in patients with non-diabetic stable coronary artery disease.
25397410 2014 Effects of increased von Willebrand factor levels on primary hemostasis in thrombocytopenic patients with liver cirrhosis.
25395424 2015 von Willebrand factor is a cofactor in complement regulation.
25390649 2014 Effects of radiofrequency catheter ablation of atrial fibrillation on soluble P-selectin, von Willebrand factor and IL-6 in the peripheral and cardiac circulation.
25387000 2015 Von Willebrand factor is elevated in individuals infected with Sudan virus and is associated with adverse clinical outcomes.
25381443 2014 Thrombin-dependent Incorporation of von Willebrand Factor into a Fibrin Network.
25363217 2014 Widespread heavy damage of capillary endothelial cells in the pathogenesis of sarcoidosis--Evidence by monoclonal von Willebrand factor immunohistochemistry in the bronchus and lung of patients with sarcoidosis.
25341886 2014 Platelet GpIba binding to von Willebrand Factor under fluid shear:contributions of the D?D3-domain, A1-domain flanking peptide and O-linked glycans.
25305202 2014 Ibrutinib treatment affects collagen and von Willebrand factor-dependent platelet functions.
25300811 2014 Towards improved diagnosis of von Willebrand disease: comparative evaluations of several automated von Willebrand factor antigen and activity assays.
25300810 2014 A two-step approach (Enzyme-linked immunosorbent assay and confirmation assay) to detect antibodies against von Willebrand factor in patients with Acquired von Willebrand Syndrome.
25274249 2015 Thrombin generation in the presence of platelets is sensitive to the activation status of von Willebrand factor.
25250329 2014 Association of vWA and TPOX polymorphisms with venous thrombosis in Mexican mestizos.
25230768 2014 Loss of cysteine 584 impairs the storage and release, but not the synthesis of von Willebrand factor.
25201004 2014 Platelet-free shear flow assay facilitates analysis of shear-dependent functions of VWF and ADAMTS13.
25196897 2014 Recombinant factor VIII Fc fusion protein: extended-interval dosing maintains low bleeding rates and correlates with von Willebrand factor levels.
25185554 2014 Misfolding of vWF to pathologically disordered conformations impacts the severity of von Willebrand disease.
25113276 2015 Imbalance of von Willebrand factor and its cleaving protease ADAMTS13 during systemic inflammation superimposed on advanced cirrhosis.
25105915 2014 Von Willebrand Factor and Factor VIII levels in Egyptian children with newly diagnosed acute lymphoblastic leukemia in relation to peripheral blast cells and steroid therapy.
25103891 2014 Storage and secretion of naturally occurring von Willebrand factor A domain variants.
25010251 2014 Interactions of von Willebrand factor and ADAMTS13 in von Willebrand disease and thrombotic thrombocytopenic purpura.
24954072 2014 von Willebrand factor in iris vasculature of glaucoma patients.
24951428 2014 Acquired von Willebrand syndrome: von Willebrand factor propeptide to von Willebrand factor antigen ratio predicts remission status.
24937073 2014 Von Willebrand factor regulation in patients with acute and chronic cerebrovascular disease: a pilot, case-control study.
24790143 2014 von Willebrand factor directly interacts with DNA from neutrophil extracellular traps.
24769235 2014 The role of von Willebrand factor as a biomarker of tumor development in hepatitis B virus-associated human hepatocellular carcinoma: a quantitative proteomic based study.
24754487 2014 Shear-stress-induced conformational changes of von Willebrand factor in a water-glycerol mixture observed with single molecule microscopy.
24740645 2014 FRETS-VWF73 rather than CBA assay reflects ADAMTS13 proteolytic activity in acquired thrombotic thrombocytopenic purpura patients.
24733925 2014 Secretion of von Willebrand factor by endothelial cells links sodium to hypercoagulability and thrombosis.
24700780 2014 Solution structure of the major factor VIII binding region on von Willebrand factor.
24681085 2014 High shear dependent von Willebrand factor self-assembly fostered by platelet interaction and controlled by ADAMTS13.
24642750 2014 Platelet adhesion involves a novel interaction between vimentin and von Willebrand factor under high shear stress.
24640675 2013 [Markers of endothelial dysfunction (VCAM-1 and vWF) in chronic hepatitis C].
24626470 2014 Performance related factors are the main determinants of the von Willebrand factor response to exhaustive physical exercise.
24558203 2014 Control of VWF A2 domain stability and ADAMTS13 access to the scissile bond of full-length VWF.
24550384 2014 Single particle tracking of ADAMTS13 (a disintegrin and metalloprotease with thrombospondin type-1 repeats) molecules on endothelial von Willebrand factor strings.
24465435 2014 Possible race and gender divergence in association of genetic variations with plasma von Willebrand factor: a study of ARIC and 1000 genome cohorts.
24449821 2014 Plasmin cleavage of von Willebrand factor as an emergency bypass for ADAMTS13 deficiency in thrombotic microangiopathy.
24441931 2014 The association between endothelial dysfunction and hypertensive retinopathy in essential hypertension.
24415745 2014 Factors associated with early platelet activation in obese children.
24406064 2014 The O-linked glycans of human von Willebrand factor modulate its interaction with ADAMTS-13.
24394662 2014 Highly reinforced structure of a C-terminal dimerization domain in von Willebrand factor.
24391089 2014 Structural basis of regulation of von Willebrand factor binding to glycoprotein Ib.
24357063 2014 Carboxyl terminus of ADAMTS13 directly inhibits platelet aggregation and ultra large von Willebrand factor string formation under flow in a free-thiol-dependent manner.
24351199 2014 Decreased von Willebrand factor ristocetin cofactor activity and increased ADAMTS13 antigen increase postoperative drainage after coronary artery bypass grafting.
24344794 2014 Changes in procoagulants track longitudinally with insulin resistance: findings from the coronary artery risk development in young adults (CARDIA) study.
24338608 2014 Von Willebrand factor: form for function.
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24270421 2013 von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin ?IIb?3.
24243921 2014 Prognostic value of plasma levels of thrombomodulin and von Willebrand factor in Egyptian children with acute lymphoblastic leukemia.
24211824 2014 Role of hemostatic factors on the risk of venous thrombosis in people with impaired kidney function.
24106205 2013 Altered glycosylation of platelet-derived von Willebrand factor confers resistance to ADAMTS13 proteolysis.
24097977 2013 Interaction of Shiga toxin with the A-domains and multimers of von Willebrand Factor.
24075746 2013 Relationship of Von Willebrand Factor with carotid artery and aortic arch calcification in ischemic stroke patients.
24062306 2013 The N-terminal flanking region of the A1 domain regulates the force-dependent binding of von Willebrand factor to platelet glycoprotein Ib?.
24056772 2013 Autophagy regulates endothelial cell processing, maturation and secretion of von Willebrand factor.
24014239 2014 The interaction between factor H and VWF increases factor H cofactor activity and regulates VWF prothrombotic status.
24010820 2013 von Willebrand factor remodeling during exocytosis from vascular endothelial cells.
24010664 2013 Exponential size distribution of von Willebrand factor.
24008159 2013 Human complement factor H is a reductase for large soluble von Willebrand factor multimers--brief report.
23991205 2013 The interaction between factor H and Von Willebrand factor.
23979707 2013 SILAC-based proteomics of human primary endothelial cell morphogenesis unveils tumor angiogenic markers.
23902764 2013 A mechanism for localized dynamics-driven affinity regulation of the binding of von Willebrand factor to platelet glycoprotein Ib?.
23834637 2013 Phenotypic and genotypic characterization of 10 Finnish patients with von Willebrand disease type 3: discovery of two main mutations.
23819767 2013 The type 2B p.R1306W natural mutation of von Willebrand factor dramatically enhances the multimer sensitivity to shear stress.
23812299 2014 Blocking von Willebrand factor for treatment of cutaneous inflammation.
23775931 2013 The linker between the D3 and A1 domains of vWF suppresses A1-GPIb? catch bonds by site-specific binding to the A1 domain.
23775583 2014 p.P2063S: a neutral VWF variant masquerading as a mutation.
23757730 2013 cAMP-induced secretion of endothelial von Willebrand factor is regulated by a phosphorylation/dephosphorylation switch in annexin A2.
23737952 2013 Terminal platelet production is regulated by von Willebrand factor.
23720451 2013 Ultralarge von Willebrand factor fibers mediate luminal Staphylococcus aureus adhesion to an intact endothelial cell layer under shear stress.
23702511 2013 Identification and characterisation of mutations associated with von Willebrand disease in a Turkish patient cohort.
23690449 2013 Common and rare von Willebrand factor (VWF) coding variants, VWF levels, and factor VIII levels in African Americans: the NHLBI Exome Sequencing Project.
23682841 2013 Normal cleavage of von Willebrand factor by ADAMTS-13 in the absence of factor VIII in patients with severe hemophilia A.
23636243 2013 Compromised shear-dependent cleavage of type 2N von Willebrand factor variants by ADAMTS13 in the presence of factor VIII.
23621778 2013 A synonymous (c.3390C>T) or a splice-site (c.3380-2A>G) mutation causes exon 26 skipping in four patients with von Willebrand disease (2A/IIE).
23539537 2013 Insights into pathological mechanisms of missense mutations in C-terminal domains of von Willebrand factor causing qualitative or quantitative von Willebrand disease.
23529929 2013 Role of RNA splicing in mediating lineage-specific expression of the von Willebrand factor gene in the endothelium.
23520336 2013 No increase in bleeding identified in type 1 VWD subjects with D1472H sequence variation.
23481506 2013 Ratio of von Willebrand factor propeptide to ADAMTS13 is associated with severity of sepsis.
23446343 2013 C2362F mutation gives rise to an ADAMTS13-resistant von Willebrand factor.
23433535 2013 Increased VWF antigen levels and decreased ADAMTS13 activity in preeclampsia.
23426949 2013 Analysis of the storage and secretion of von Willebrand factor in blood outgrowth endothelial cells derived from patients with von Willebrand disease.
23414517 2013 A human skeletal muscle interactome centered on proteins involved in muscular dystrophies: LGMD interactome.
23407766 2013 Phenotypic and molecular characterisation of type 3 von Willebrand disease in a cohort of Indian patients.
23383177 2013 The oxidative modification of von Willebrand factor is associated with thrombotic angiopathies in diabetes mellitus.
23376485 2013 Proteomic analysis of podocyte exosome-enriched fraction from normal human urine.
23358615 2013 Changes of plasma vWF level in response to the improvement of air quality: an observation of 114 healthy young adults.
23355534 2013 Cellular and molecular basis of von Willebrand disease: studies on blood outgrowth endothelial cells.
23354996 2013 A common founder mutation p.P2063S in exon 36 of VWF in 11 unrelated Indian von Willebrand disease (VWD) families.
23349392 2013 VWF propeptide and ratios between VWF, VWF propeptide, and FVIII in the characterization of type 1 von Willebrand disease.
23335371 2013 Mutations in the A3 domain of von Willebrand factor inducing combined qualitative and quantitative defects in the protein.
23311757 2013 The genetics of Canadian type 3 von Willebrand disease: further evidence for co-dominant inheritance of mutant alleles.
23267103 2013 Linkage analysis identifies a locus for plasma von Willebrand factor undetected by genome-wide association.
23266519 2013 Behavior of ADAMTS13 and Von Willebrand factor levels in patients after living donor liver transplantation.
23216583 2013 Characterizing polymorphisms and allelic diversity of von Willebrand factor gene in the 1000 Genomes.
23179108 2013 Characterisation of mutations and molecular studies of type 2 von Willebrand disease.
23168412 2013 Distinct roles of Ser-764 and Lys-773 at the N terminus of von Willebrand factor in complex assembly with coagulation factor VIII.
23104847 2013 Direct observation of von Willebrand factor elongation and fiber formation on collagen during acute whole blood exposure to pathological flow.
23041092 2013 Prognostic value of plasma von Willebrand factor and its cleaving protease ADAMTS13 in patients with atrial fibrillation.
23020229 2012 Smoking, von Willebrand factor and ADAMTS-13 in healthy males.
23006049 2012 Polyphosphate binds to human von Willebrand factor in vivo and modulates its interaction with glycoprotein Ib.
22982001 2012 Association between variations in coagulation system genes and carotid plaque.
22972845 2013 Comparative study on the histomorphology and molecular biology of radial artery conduits in patients with diabetes mellitus who underwent coronary bypass surgery.
22952809 2012 PR3 and elastase alter PAR1 signaling and trigger vWF release via a calcium-independent mechanism from glomerular endothelial cells.
22923007 2012 Correlation of von Willebrand factor gene polymorphism and coronary heart disease.
22922961 2012 Simultaneous exposure of sites in von Willebrand factor for glycoprotein Ib binding and ADAMTS13 cleavage: studies with ristocetin.
22896002 2012 Clinical and prognostic implications of low or high level of von Willebrand factor in patients with Waldenstrom macroglobulinemia.
22871923 2012 Mutation distribution in the von Willebrand factor gene related to the different von Willebrand disease (VWD) types in a cohort of VWD patients.
22854959 2012 Light chain of factor VIII is sufficient for accelerating cleavage of von Willebrand factor by ADAMTS13 metalloprotease.
22849435 2012 Mapping the N-glycome of human von Willebrand factor.
22812646 2012 Shear stress is required for the endocytic uptake of the factor VIII-von Willebrand factor complex by macrophages.
22781599 2012 [Increased susceptibility of recombinant type 2A von Willebrand factor mutant A1500E to proteolysis by ADAMTS13].
22740102 2012 The anti-von Willebrand factor aptamer ARC1779 increases von Willebrand factor levels and platelet counts in patients with type 2B von Willebrand disease.
22718838 2012 The B subunits of Shiga-like toxins induce regulated VWF secretion in a phospholipase D1-dependent manner.
22688555 2012 Effect of glucocorticoids on von Willebrand factor levels and its correlation with von Willebrand factor gene promoter polymorphism.
22674667 2012 Molecular characterization, recombinant protein expression, and mRNA analysis of type 3 von Willebrand disease: Studies of an Italian cohort of 10 patients.
22612323 2012 Oxidative damage to pre-eclamptic placenta: immunohistochemical expression of VEGF, nitrotyrosine residues and von Willebrand factor.
22596213 2012 Baseline vWF factor predicts the development of elevated pulmonary artery pressure in systemic sclerosis.
22593709 2012 Endothelial cell markers in patients with pseudoexfoliation syndrome.
22568520 2012 von Willebrand factor plasma levels, genetic variations and coronary heart disease in an older population.
22559004 2012 Thermodynamic analysis of the interaction of factor VIII with von Willebrand factor.
22545467 2012 [The endothelin-1 and von Willebrand factor in the development of lung hypertension in children with chronic bronchopulmonary pathology].
22537243 2012 Characterisation of von Willebrand factor A1 domain mutants I1416N and I1416T: correlation of clinical phenotype with flow-based platelet adhesion.
22532296 2012 von Willebrand factor as new noninvasive predictor of portal hypertension, decompensation and mortality in patients with liver cirrhosis.
22517896 2012 O-linked glycosylation of von Willebrand factor modulates the interaction with platelet receptor glycoprotein Ib under static and shear stress conditions.
22507806 2012 Von Willebrand factor (VWF) as a risk factor for bleeding and thrombosis.
22507569 2012 Critical von Willebrand factor A1 domain residues influence type VI collagen binding.
22503077 2012 Nonsense mediated decay of VWF mRNA subsequent to c.7674-7675insC mutation in type3 VWD patients.
22490677 2012 Sequence and structure relationships within von Willebrand factor.
22487084 2012 Differential sensitivity of von Willebrand factor (VWF) 'activity' assays to large and small VWF molecular weight forms: a cross-laboratory study comparing ristocetin cofactor, collagen-binding and mAb-based assays.
22479377 2012 Identification and functional analysis of a novel von Willebrand factor mutation in a family with type 2A von Willebrand disease.
22477538 2012 von Willebrand factor: at the crossroads of bleeding and thrombosis.
22466813 2012 Relationship between ABO blood groups and von Willebrand factor, ADAMTS13 and factor VIII in patients undergoing hemodialysis.
22452980 2012 von Willebrand factor (VWF) propeptide binding to VWF D'D3 domain attenuates platelet activation and adhesion.
22431729 2012 N-terminal flanking region of A1 domain in von Willebrand factor stabilizes structure of A1A2A3 complex and modulates platelet activation under shear stress.
22431572 2012 Intersection of mechanisms of type 2A VWD through defects in VWF multimerization, secretion, ADAMTS-13 susceptibility, and regulated storage.
22429825 2012 Reduced von Willebrand factor secretion is associated with loss of Weibel-Palade body formation.
22370688 von Willebrand factor, shear stress, and ADAMTS13 in hemostasis and thrombosis.
22357761 2012 Calcium stabilizes the von Willebrand factor A2 domain by promoting refolding.
22298244 2012 ADAMTS13 and Von Willebrand factor in patients undergoing hemodialysis.
22282355 2012 ARC15105 is a potent antagonist of von Willebrand factor mediated platelet activation and adhesion.
22268915 2012 von Willebrand factor propeptide to antigen ratio in acquired thrombotic thrombocytopenic purpura.
22267483 2012 Identification of galectin-1 and galectin-3 as novel partners for von Willebrand factor.
22257027 2012 Genetic determinants of von Willebrand factor plasma levels and the risk of stroke: the Rotterdam Study.
22244714 2013 von Willebrand factor genetic variant associated with hematoma expansion after intracerebral hemorrhage.
22219226 2012 Influence of single nucleotide polymorphisms in factor VIII and von Willebrand factor genes on plasma factor VIII activity: the ARIC Study.
22207689 2012 Biogenesis of Weibel-Palade bodies in von Willebrand's disease variants with impaired von Willebrand factor intrachain or interchain disulfide bond formation.
22197721 2012 VWF mutations and new sequence variations identified in healthy controls are more frequent in the African-American population.
22178067 2012 Modulation of the von Willebrand factor-dependent platelet adhesion through alternative proteolytic pathways.
22163125 2011 The role of von Willebrand factor and ADAMTS13 in the no-reflow phenomenon: after primary percutaneous coronary intervention.
22139419 2011 New gene functions in megakaryopoiesis and platelet formation.
22125593 2011 Elevated plasma von Willebrand factor and propeptide levels in Malawian children with malaria.
22110247 2012 High VWF, low ADAMTS13, and oral contraceptives increase the risk of ischemic stroke and myocardial infarction in young women.
22100896 2012 Soluble CD40 ligand, soluble P-selectin and von Willebrand factor levels in subjects with prediabetes: the impact of metabolic syndrome.
22096550 2011 Temperature-dependence of Weibel-Palade body exocytosis and cell surface dispersal of von Willebrand factor and its propolypeptide.
22091998 2012 Oxidation of Met1606 in von Willebrand factor is a risk factor for thrombotic and septic complications in chronic renal failure.
22077817 2012 Long-term expression of human coagulation factor VIII in a tolerant mouse model using the ?C31 integrase system.
22070827 2012 Plasma ADAMTS13, von Willebrand factor (VWF) and VWF propeptide profiles in patients with DIC and related diseases.
22056526 2012 Coating conditions matter to collagen matrix formation regarding von Willebrand factor and platelet binding.
22032085 2011 Interpretation of correlations between coagulation factors FV, FVIII and vWF in normal and type 2 diabetes mellitus patients.
22004444 2012 A family with type 2M VWD with normal VWF:RCo but reduced VWF:CB and a M1761K mutation in the A3 domain.
21940821 2011 Factor VIII alters tubular organization and functional properties of von Willebrand factor stored in Weibel-Palade bodies.
21937160 2012 Modeling ADAMTS13-von Willebrand factor interaction: Implications for oxidative stress-related cardiovascular diseases and type 2A von Willebrand disease.
21917758 2011 Shear stress-induced unfolding of VWF accelerates oxidation of key methionine residues in the A1A2A3 region.
21911836 2011 Lateral self-association of VWF involves the Cys2431-Cys2453 disulfide/dithiol in the C2 domain.
21909423 2011 Cleavage of von Willebrand factor by granzyme M destroys its factor VIII binding capacity.
21904649 2011 Von Willebrand factor and oxidative stress parameters in acute coronary syndromes.
21896483 2011 Local elongation of endothelial cell-anchored von Willebrand factor strings precedes ADAMTS13 protein-mediated proteolysis.
21857647 2011 A pH-regulated dimeric bouquet in the structure of von Willebrand factor.
21847508 2011 Immunohistochemical expression of von Willebrand factor in the preeclamptic placenta.
21844207 2011 Actomyosin II contractility expels von Willebrand factor from Weibel-Palade bodies during exocytosis.
21839029 2011 Role of von Willebrand factor in the haemostasis.
21831097 2011 Value assignment of the WHO 2nd International Standard von Willebrand factor, concentrate (09/182).
21810271 2011 Combined analysis of three genome-wide association studies on vWF and FVIII plasma levels.
21792464 2011 [Von Willebrand factor and ADAMTS13 balancing primary haemostasis].
21779388 2011 A conformation-sensitive monoclonal antibody against the A2 domain of von Willebrand factor reduces its proteolysis by ADAMTS13.
21729570 2011 [Analysis of vWF gene A1381T polymorphism in patients with coronary heart disease].
21715306 2011 Unraveling the scissile bond: how ADAMTS13 recognizes and cleaves von Willebrand factor.
21711445 2011 Expanded phenotype-genotype correlations in a pediatric population with type 1 von Willebrand disease.
21705658 2011 Mechanism of von Willebrand factor scissile bond cleavage by a disintegrin and metalloproteinase with a thrombospondin type 1 motif, member 13 (ADAMTS13).
21638248 2011 Expression of P-selectin, von Willebrand and endothelin-1 after carotid artery stenting.
21629201 2011 The relationship between levels of von Willebrand factor and mean platelet volume in subjects with isolated impaired fasting glucose.
21605335 2011 Oxidized von Willebrand factor is efficiently cleaved by serine proteases from primary granules of leukocytes: divergence from ADAMTS-13.
21596755 2011 Intracellular storage and regulated secretion of von Willebrand factor in quantitative von Willebrand disease.
21592973 2011 Phylogenetic and functional analysis of histidine residues essential for pH-dependent multimerization of von Willebrand factor.
21592258 2012 C1272F: a novel type 2A von Willebrand's disease mutation in A1 domain; its clinical significance.
21518247 2011 Relationship between thrombospondin gene variations, von Willebrand factor levels and the risk of coronary heart disease in an older population.
21512165 2011 Von Willebrand factor and ADAMTS13: a candidate couple for preeclampsia pathophysiology.
21507943 2011 Platelet glycoprotein Ib beta/IX mediates glycoprotein Ib alpha localization to membrane lipid domain critical for von Willebrand factor interaction at high shear.
21474661 2011 Differential roles of fibrinogen and von Willebrand factor on clot formation and platelet adhesion in reconstituted and immune thrombocytopenia.
21471547 Endothelial activation markers soluble E-selectin and von Willebrand factor in primary hyperparathyroidism.
21410641 2011 Large deletions identified in patients with von Willebrand disease using multiple ligation-dependent probe amplification.
21393328 2011 An apparently silent nucleotide substitution (c.7056C>T) in the von Willebrand factor gene is responsible for type 1 von Willebrand disease.
21362127 2011 Common large partial VWF gene deletion does not cause alloantibody formation in the Hungarian type 3 von Willebrand disease population.
21352469 2011 Decreased active von Willebrand factor level owing to shear stress in aortic stenosis patients.
21346256 2011 Pathologic mechanisms of type 1 VWD mutations R1205H and Y1584C through in vitro and in vivo mouse models.
21343614 2011 Genetic determinants of plasma von Willebrand factor antigen levels: a target gene SNP and haplotype analysis of ARIC cohort.
21342431 2011 Genetic determinants of von Willebrand factor levels and activity in relation to the risk of cardiovascular disease: a review.
21304226 2011 New insight into the hypercoagulability of Cushing's syndrome.
21266777 2011 N-acetylcysteine reduces the size and activity of von Willebrand factor in human plasma and mice.
21174003 2011 Inhibition of von Willebrand factor by ARC1779 in patients with acute thrombotic thrombocytopenic purpura.
21163921 2011 Genetic variation associated with plasma von Willebrand factor levels and the risk of incident venous thrombosis.
21150180 2011 Thrombomodulin and von Willebrand factor: relation to endothelial dysfunction and disease outcome in children with acute lymphoblastic leukemia.
21143374 2011 Effect of the VWF promoter (GT)n repeat and single-nucleotide polymorphism c.-2527G>A on circulating von Willebrand factor levels under normal conditions.
21136020 2011 Levels of von Willebrand factor and ADAMTS13 determine clinical outcome after cardioversion for atrial fibrillation.
21094987 2011 A new method measuring the interaction between von Willebrand factor and coagulation factor VIII.
21083900 2010 Von willebrand factor increases endothelial cell adhesiveness for human mesenchymal stem cells by activating p38 mitogen-activated protein kinase.
21048155 2011 Endothelial von Willebrand factor regulates angiogenesis.
21044367 2010 An integrative method for scoring candidate genes from association studies: application to warfarin dosing.
21037087 2011 Key role of glycoprotein Ib/V/IX and von Willebrand factor in platelet activation-dependent fibrin formation at low shear flow.
21029358 2011 Impact on venous thrombosis risk of newly discovered gene variants associated with FVIII and VWF plasma levels.
20979592 2010 Redox control of ?2-glycoprotein I-von Willebrand factor interaction by thioredoxin-1.
20958794 2011 VWF and ADAMTS13 behavior in estradiol-treated HUVEC.
20946172 2010 Disulfide bond reduction of von Willebrand factor by ADAMTS-13.
20941784 2010 A common variant in the Von Willebrand factor gene is associated with multiple functional consequences.
20940418 2011 Variation in the von Willebrand factor gene is associated with von Willebrand factor levels and with the risk for cardiovascular disease.
20854374 2010 Alterations of mRNA processing and stability as a pathogenic mechanism in von Willebrand factor quantitative deficiencies.
20851871 2010 Polymorphic variation within the VWF gene contributes to the failure to detect mutations in patients historically diagnosed with type 1 von Willebrand disease from the MCMDM-1VWD cohort.
20806123 2010 Evaluation of commercial von Willebrand factor collagen binding assays to assist the discrimination of types 1 and 2 von Willebrand disease.
20806106 2010 Comparative study of coagulation and thrombin generation in the portal and jugular plasma of patients with cirrhosis.
20737318 2010 [Effect of von Willebrand factor on the biological characteristics of colorectal cancer cells].
20729548 2010 Collagen VI microfibril formation is abolished by an {alpha}2(VI) von Willebrand factor type A domain mutation in a patient with Ullrich congenital muscular dystrophy.
20725043 2010 A mechanically stabilized receptor-ligand flex-bond important in the vasculature.
20723310 2010 [Impact of vWF gene A1381T polymorphism and ABO blood group on von Willebrand factor level in plasma].
20705333 2010 Thrombospondin-1 and ADAMTS13 competitively bind to VWF A2 and A3 domains in vitro.
20704649 2010 von Willebrand factor clearance does not involve proteolysis by ADAMTS-13.
20701764 2010 Neuropeptide delivery to the brain: a von Willebrand factor signal peptide to direct neuropeptide secretion.
20696945 2010 Functional characterization of a 13-bp deletion (c.-1522_-1510del13) in the promoter of the von Willebrand factor gene in type 1 von Willebrand disease.
20696943 2010 von Willebrand factor self-association on platelet GpIbalpha under hydrodynamic shear: effect on shear-induced platelet activation.
20695979 2010 The distal carboxyterminal domains of murine ADAMTS13 influence proteolysis of platelet-decorated VWF strings in vivo.
20682599 2011 Polymorphisms and mutations in vWF and ADAMTS13 genes and their correlation with plasma levels of FVIII and vWF in patients with deep venous thrombosis.
20675279 2010 The endothelial cell markers von Willebrand Factor (vWF), CD31 and CD34 are lost in glomerulonephritis and no longer correlate with the morphological indices of glomerular sclerosis, interstitial fibrosis, activity and chronicity.
20673868 2010 A genetic association study of maternal and fetal candidate genes that predispose to preterm prelabor rupture of membranes (PROM).
20651279 2010 Relationships between sleep duration and von Willebrand factor, factor VII, and fibrinogen: Whitehall II study.
20650506 2010 Validation of a rapid test (VWF-LIA) for the quantitative determination of von Willebrand factor antigen in type 1 von Willebrand disease diagnosis within the European multicenter study MCMDM-1VWD.
20628086 2010 Variation at the NFATC2 locus increases the risk of thiazolidinedione-induced edema in the Diabetes REduction Assessment with ramipril and rosiglitazone Medication (DREAM) study.
20627205 2010 Sources of variation in factor VIII, von Willebrand factor and fibrinogen measurements: implications for detecting deficiencies and increased plasma levels.
20605782 2010 Factor VIII and platelets synergistically accelerate cleavage of von Willebrand factor by ADAMTS13 under fluid shear stress.
20589320 2010 mRNA levels of CD31, CD144, CD146 and von Willebrand factor do not serve as surrogate markers for circulating endothelial cells.
20589314 2010 Comparison of plasma-derived and recombinant von Willebrand factor by atomic force microscopy.
20589313 2010 A randomised pilot trial of the anti-von Willebrand factor aptamer ARC1779 in patients with type 2b von Willebrand disease.
20586924 2010 Homozygous type 2N R854W von Willebrand factor is poorly secreted and causes a severe von Willebrand disease phenotype.
20570857 2010 The dominant-negative von Willebrand factor gene deletion p.P1127_C1948delinsR: molecular mechanism and modulation.
20565774 2010 Population based allele frequencies of disease associated polymorphisms in the Personalized Medicine Research Project.
20561182 2010 Blood group significantly influences von Willebrand factor increase and half-life after desmopressin in von Willebrand disease Vicenza.
20508517 2010 Increased serum vWF and sVCAM-1 levels are associated with late or very late angiographic stent thrombosis after sirolimus-eluting stent implantation.
20505748 2010 The mechanism of melanoma-associated thrombin activity and von Willebrand factor release from endothelial cells.
20498367 2010 Destabilization of the A1 domain in von Willebrand factor dissociates the A1A2A3 tri-domain and provokes spontaneous binding to glycoprotein Ibalpha and platelet activation under shear stress.
20492463 2010 von Willebrand factor variant p.Arg924Gln marks an allele associated with reduced von Willebrand factor and factor VIII levels.
20491956 2010 A novel alanine or threonine 789 to proline mutation causing type 2N von Willebrand's disease when inherited homozygously or heterozygously with arginine 854 to glutamine mutation.
20452482 2010 Identification of fetal and maternal single nucleotide polymorphisms in candidate genes that predispose to spontaneous preterm labor with intact membranes.
20431063 2010 Repressors NFI and NFY participate in organ-specific regulation of von Willebrand factor promoter activity in transgenic mice.
20424473 2010 L-type voltage-dependent calcium channel alpha subunit 1C is a novel candidate gene associated with secondary hyperparathyroidism: an application of haplotype-based analysis for multiple linked single nucleotide polymorphisms.
20418283 2010 Specific N-linked glycosylation sites modulate synthesis and secretion of von Willebrand factor.
20403097 2010 Functional variation in the arginine vasopressin 2 receptor as a modifier of human plasma von Willebrand factor levels.
20354169 2010 The importance of vicinal cysteines, C1669 and C1670, for von Willebrand factor A2 domain function.
20351307 2010 A cluster of mutations in the D3 domain of von Willebrand factor correlates with a distinct subgroup of von Willebrand disease: type 2A/IIE.
20346360 2010 Genetic risk factors for hepatopulmonary syndrome in patients with advanced liver disease.
20335223 2010 The mutation N528S in the von Willebrand factor (VWF) propeptide causes defective multimerization and storage of VWF.
20303469 2010 Type 1 von Willebrand disease due to reduced von Willebrand factor synthesis and/or survival: observations from a case series.
20231535 2010 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.
20231421 2010 Common VWF exon 28 polymorphisms in African Americans affecting the VWF activity assay by ristocetin cofactor.
20218000 1983 Potentiation of wheat germ agglutinin aggregation of platelets by von Willebrand protein and by a 116,000 molecular weight tryptic fragment.
20201926 2010 Human variation in alcohol response is influenced by variation in neuronal signaling genes.
20197612 2010 [Change of HIF-1alpha protein expression in the placenta bed and concentration of vWF in maternal peripheral blood of pre-eclampsia].
20156642 2010 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.
20142117 2010 Efficacy of simvastatin or ezetimibe on tissue factor, von Willebrand's factor and C-reactive protein in patients with hypercholesterolaemia.
20118404 2010 Abnormal VWF modifies megakaryocytopoiesis: studies of platelets and megakaryocyte cultures from patients with von Willebrand disease type 2B.
20075158 2010 Amino acid residues Arg(659), Arg(660), and Tyr(661) in the spacer domain of ADAMTS13 are critical for cleavage of von Willebrand factor.
20063990 2010 Are integrin alpha(2)beta(1), glycoprotein Ib and vWf levels correlated with their contributions to platelet adhesion on collagen under high-shear flow?
20062916 2010 Severe malaria is associated with a deficiency of von Willebrand factor cleaving protease, ADAMTS13.
20060388 2010 VWF pseudogene: Mimics, masks and spoils.
20058209 2010 Pathophysiology of thrombotic thrombocytopenic purpura.
20036902 2010 Hemostatic gene polymorphisms in young Sardinian with non-fatal acute myocardial infarction.
20032502 2010 An autoantibody epitope comprising residues R660, Y661, and Y665 in the ADAMTS13 spacer domain identifies a binding site for the A2 domain of VWF.
20030949 2009 [Polymorphism of DXS15, CA13, CA22 loci in Guangdong normal population].
20024497 2010 Proteolytic resistance conferred to fibrinogen by von Willebrand factor.
20021313 2009 Impaired glucose tolerance and endothelial damage, as assessed by levels of von Willebrand factor and circulating endothelial cells, following acute myocardial infarction.
19965639 2010 Expression of terminal alpha2-6-linked sialic acid on von Willebrand factor specifically enhances proteolysis by ADAMTS13.
19948975 2009 Integrative predictive model of coronary artery calcification in atherosclerosis.
19944670 2010 Identification of ADAMTS13 peptide sequences binding to von Willebrand factor.
19943880 2010 Relevance of chloride binding to von Willebrand factor in type 2B von Willebrand disease patients.
19933990 2010 Accumulation of gene polymorphisms related to plaque disruption and thrombosis is associated with cerebral infarction in subjects with type 2 diabetes.
19913121 2009 Gene-centric association signals for lipids and apolipoproteins identified via the HumanCVD BeadChip.
19897584 2010 Force-induced cleavage of single VWFA1A2A3 tridomains by ADAMTS-13.
19897581 2010 Rapid activation of endothelial cells enables Plasmodium falciparum adhesion to platelet-decorated von Willebrand factor strings.
19875727 2009 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.
19874459 2010 The plasma von Willebrand factor O-glycome comprises a surprising variety of structures including ABH antigens and disialosyl motifs.
19840363 2010 The glycoprotein Ibalpha-von Willebrand factor interaction induces platelet apoptosis.
19839997 2009 Similar immune profile in bipolar disorder and schizophrenia: selective increase in soluble tumor necrosis factor receptor I and von Willebrand factor.
19820200 2009 Shear-induced interaction of platelets with von Willebrand factor results in glycoprotein Ibalpha shedding.
19817991 2009 Shear-induced unfolding activates von Willebrand factor A2 domain for proteolysis.
19812385 2010 Oxidative modification of von Willebrand factor by neutrophil oxidants inhibits its cleavage by ADAMTS13.
19773258 2010 Premature termination codon mutations in the von Willebrand factor gene are associated with allele-specific and position-dependent mRNA decay.
19765213 2009 Nitric oxide inhibits von Willebrand factor-mediated platelet adhesion and spreading through regulation of integrin alpha(IIb)beta(3) and myosin light chain.
19765212 2009 Multi-step binding of ADAMTS-13 to von Willebrand factor.
19740526 2010 A family having type 2B von Willebrand disease with an R1306W mutation: Severe thrombocytopenia leads to the normalization of high molecular weight multimers.
19732980 2010 Baseline von Willebrand factor plasma levels and no-reflow phenomenon after primary percutaneous coronary intervention for ST segment elevation myocardial infarction.
19730683 2009 The variant rs1867277 in FOXE1 gene confers thyroid cancer susceptibility through the recruitment of USF1/USF2 transcription factors.
19729451 2009 ADAMTS13 bound to endothelial cells exhibits enhanced cleavage of von Willebrand factor.
19726719 2009 Glycoprotein Ibalpha inhibitor complex structure reveals a combined steric and allosteric mechanism of von Willebrand factor antagonism.
19718479 2009 Levels of von Willebrand factor antigen and von Willebrand factor cleaving protease (ADAMTS13) activity predict clinical events in chronic heart failure.
19718474 2009 A G-quartet oligonucleotide blocks glycoprotein Ib-mediated platelet adhesion and aggregation under flow conditions.
19687512 2009 Characterization of W1745C and S1783A: 2 novel mutations causing defective collagen binding in the A3 domain of von Willebrand factor.
19682236 2009 von Willebrand factor cleaved from endothelial cells by ADAMTS13 remains ultralarge in size.
19647361 2009 Kinetic study of von Willebrand factor self-aggregation induced by ristocetin.
19630816 2009 Altered megakaryocytopoiesis in von Willebrand type 2B disease.
19630761 2009 von Willebrand factor assembly and secretion.
19624459 2009 An assessment of the pathogenic significance of the R924Q von Willebrand factor substitution.
19608674 2009 Platelet morphological changes in 2 patients with von Willebrand disease type 3 caused by large homozygous deletions of the von Willebrand factor gene.
19587373 2009 A novel binding site for ADAMTS13 constitutively exposed on the surface of globular VWF.
19570985 2009 Vascular endothelial growth factor (VEGF) receptor-2 tyrosine 1175 signaling controls VEGF-induced von Willebrand factor release from endothelial cells via phospholipase C-gamma 1- and protein kinase A-dependent pathways.
19566550 2009 Expression of 14 von Willebrand factor mutations identified in patients with type 1 von Willebrand disease from the MCMDM-1VWD study.
19541819 2009 Leukocyte proteases cleave von Willebrand factor at or near the ADAMTS13 cleavage site.
19506362 2009 Dominant von Willebrand disease type 2A groups I and II due to missense mutations in the A2 domain of the von Willebrand factor gene: diagnosis and management.
19506361 2009 Dominant von Willebrand disease type 2M and 2U are variable expressions of one distinct disease entity caused by loss-of-function mutations in the A1 domain of the von Willebrand factor gene.
19506360 2009 Autosomal dominant von Willebrand disease type 2M.
19506359 2009 Laboratory diagnosis of von Willebrand disease type 1/2E (2A subtype IIE), type 1 Vicenza and mild type 1 caused by mutations in the D3, D4, B1-B3 and C1-C2 domains of the von Willebrand factor gene. Role of von Willebrand factor multimers and the von Willebrand factor propeptide/antigen ratio.
19506358 2009 Recessive von Willebrand disease type 2 Normandy: variable expression of mild hemophilia and VWD type 1.
19506357 2009 Laboratory and molecular characteristics of recessive von Willebrand disease type 2C (2A subtype IIC) of variable severity due to homozygous or double heterozygous mutations in the D1 and D2 domains.
19506356 2009 Autosomal recessive von Willebrand disease type 1 or 2 due to homozygous or compound heterozygous mutations in the von Willebrand factor gene. A single center experience on molecular heterogeneity and laboratory features in 12 families.
19506355 2009 Factor VIII-von Willebrand factor binding defects in autosomal recessive von Willebrand disease type Normandy and in mild hemophilia A. New insights into factor VIII-von Willebrand factor interactions.
19506354 2009 C1584: effect on von Willebrand factor proteolysis and von Willebrand factor antigen levels.
19506353 2009 Laboratory diagnosis and molecular basis of mild von Willebrand disease type 1.
19506352 2009 Laboratory diagnosis and molecular classification of von Willebrand disease.
19498171 2009 Mechanoenzymatic cleavage of the ultralarge vascular protein von Willebrand factor.
19496923 2009 A +220 GATA motif mediates basal but not endotoxin-repressible expression of the von Willebrand factor promoter in Hprt-targeted transgenic mice.
19477487 2009 Kynurenines and oxidative status are independently associated with thrombomodulin and von Willebrand factor levels in patients with end-stage renal disease.
19470641 2009 Structural specializations of A2, a force-sensing domain in the ultralarge vascular protein von Willebrand factor.
19436051 2009 Valves of the deep venous system: an overlooked risk factor.
19422453 2009 Type 2A (IIH) von Willebrand disease is due to mutations that affect von Willebrand factor multimerization.
19422343 2009 Development of a severe von Willebrand factor/ADAMTS13 dysbalance during orthotopic liver transplantation.
19420105 2009 A candidate gene approach to genetic prognostic factors of IgA nephropathy--a result of Polymorphism REsearch to DIstinguish genetic factors Contributing To progression of IgA Nephropathy (PREDICT-IgAN).
19372260 2009 A novel deletion mutation is recurrent in von Willebrand disease types 1 and 3.
19350125 2009 Heyde's syndrome without a decrease in large von Willebrand factor multimers: a case of intestinal bleedings reversed by valve replacement in a patient with aortic stenosis.
19346881 2009 A novel type 2A von Willebrand factor mutation (V1499E) associated with variable clinical expression.
19298374 2009 Measurement of von Willebrand factor-FVIII binding activity in patients with suspected von Willebrand disease type 2N: application of an ELISA-based assay in a reference laboratory.
19289057 2009 Fluid shear induces conformation change in human blood protein von Willebrand factor in solution.
19286880 2009 Autosomal dominant C1149R von Willebrand disease: phenotypic findings and their implications.
19277422 2009 Rapid molecular diagnosis of von Willebrand disease by direct sequencing. Detection of 12 novel putative mutations in VWF gene.
19192112 2009 Genetic alteration of the D2 domain abolishes von Willebrand factor multimerization and trafficking into storage.
19190814 2009 Increased plasma von Willebrand factor antigen levels but normal von Willebrand factor cleaving protease (ADAMTS13) activity in preeclampsia.
19190813 2009 Microsatellite (GT)(n) repeats and SNPs in the von Willebrand factor gene promoter do not influence circulating von Willebrand factor levels under normal conditions.
19190805 2009 Variations in the ratio between von Willebrand factor and its cleaving protease during systemic inflammation and association with severity and prognosis of organ failure.
19175495 2009 Platelet adhesion to multimerin 1 in vitro: influences of platelet membrane receptors, von Willebrand factor and shear.
19159218 2009 Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry.
19139490 2009 A strategy for precise and large scale identification of core fucosylated glycoproteins.
19110358 2009 Inflammation, hemostasis, and the risk of kidney function decline in the Atherosclerosis Risk in Communities (ARIC) Study.
19088379 2009 Intracellular cotrafficking of factor VIII and von Willebrand factor type 2N variants to storage organelles.
19060241 2009 The Montreal platelet syndrome kindred has type 2B von Willebrand disease with the VWF V1316M mutation.
19056867 2009 Large-scale proteomics and phosphoproteomics of urinary exosomes.
19029465 2008 Von Willebrand factor, type 2 diabetes mellitus, and risk of cardiovascular disease: the framingham offspring study.
19021456 2009 Association between von Willebrand factor gene polymorphism and preeclampsia.
18996572 2009 Changes in plasma von Willebrand factor and ADAMTS13 levels associated with left atrial remodeling in atrial fibrillation.
18983500 2009 ADAMTS-13 cleaves long von Willebrand factor multimeric strings anchored to endothelial cells in the absence of flow, platelets or conformation-altering chemicals.
18983499 2009 The influence of von Willebrand factor on factor VIII activity measurements.
18981290 2009 N-Glycans of ADAMTS13 modulate its secretion and von Willebrand factor cleaving activity.
18945966 2009 Phospholipase D1 is specifically required for regulated secretion of von Willebrand factor from endothelial cells.
18927433 2009 Integrin alpha(v)beta(3) on human endothelial cells binds von Willebrand factor strings under fluid shear stress.
18923835 2009 Impact of 789Ala/Ala genotype on quantitative type of von Willebrand disease.
18855616 2008 Thrombospondin-1 in von Willebrand factor function.
18848323 2009 Prothrombotic genetic variants and atherosclerosis in patients with cerebral ischemia of arterial origin.
18841300 2008 Is VWF R924Q a benign polymorphism, a marker of a null allele or a factor VIII-binding defect? The debate continues with results from the UKHCDO VWD study.
18809794 2008 Functional impairment of von Willebrand factor in hypertrophic cardiomyopathy: relation to rest and exercise obstruction.
18776767 2008 Type 2B von Willebrand disease associated with the release of platelet agglutinates from megakaryocytes in the bone marrow.
18771467 2008 Elevation of serum von Willebrand factor and anti-endothelial cell antibodies in patients with immunoglobulin A nephropathy are associated with intrarenal arterial lesions.
18761720 2008 The relationship of circulating endothelial cells to plasma indices of endothelial damage/dysfunction and apoptosis in acute coronary syndromes: implications for prognosis.
18752579 2008 Protein kinase C-delta mediates von Willebrand factor secretion from endothelial cells in response to vascular endothelial growth factor (VEGF) but not histamine.
18725999 2008 Platelet glycoprotein Ibalpha forms catch bonds with human WT vWF but not with type 2B von Willebrand disease vWF.
18724972 2008 Sex differences in environmental and genetic factors for hypertension.
18712522 2009 Novel missense mutation c.2685G>C (p.Q895H) in VWF gene associated with very low levels of VWF mRNA.
18690339 2008 Characterization of a novel mutation in the von Willebrand factor propeptide in a distinct subtype of recessive von Willebrand disease.
18665926 2008 An Alu-mediated novel large deletion is the most frequent cause of type 3 von Willebrand disease in Hungary.
18647229 2008 The membrane-proximal intermolecular disulfide bonds in glycoprotein Ib influence receptor binding to von Willebrand factor.
18637125 2008 N1421K mutation in the glycoprotein Ib binding domain impairs ristocetin- and botrocetin-mediated binding of von Willebrand factor to platelets.
18618671 2009 Genetic predictors of depressive symptoms in cardiac patients.
18612539 2008 Effects of von Willebrand factor concentration and platelet collision on shear-induced platelet activation.
18600088 2008 Alpha 2A adrenergic receptor polymorphism is associated with plasma von Willebrand factor levels in a general population.
18577516 2008 Antihemostatic activity of human granzyme B mediated by cleavage of von Willebrand factor.
18549909 2008 The role of VWF in the immunogenicity of FVIII.
18535282 2008 Hemostatic and inflammatory risk factors for intracerebral hemorrhage in a pooled cohort.
18521510 2008 Role of membrane cholesterol in platelet calcium signalling in response to VWF and collagen under stasis and flow.
18521502 2008 Distribution of von Willebrand factor levels in young women with and without bleeding symptoms: influence of ABO blood group and promoter haplotypes.
18515885 2008 Aquaporin 2 gene variations, risk of venous thrombosis and plasma levels of von Willebrand factor and factor VIII.
18503632 2008 Extracellular control of VWF multimer size and thiol-disulfide exchange.
18492805 2008 Factor VIII accelerates proteolytic cleavage of von Willebrand factor by ADAMTS13.
18485092 2008 Influence of a GT repeat element on shear stress responsiveness of the VWF gene promoter.
18449422 2008 Survival of von Willebrand factor released following DDAVP in a type 1 von Willebrand disease cohort: influence of glycosylation, proteolysis and gene mutations.
18433458 2008 Inflammatory cytokines inhibit ADAMTS13 synthesis in hepatic stellate cells and endothelial cells.
18433456 2008 Covalent regulation of ULVWF string formation and elongation on endothelial cells under flow conditions.
18388497 2008 Persistent factor VIII-dependent factor X activation on endothelial cells is independent of von Willebrand factor.
18347221 2008 von Willebrand factor in cardiovascular disease: focus on acute coronary syndromes.
18344423 2008 Basal secretion of von Willebrand factor from human endothelial cells.
18327408 2008 Magnesium maintains endothelial integrity, up-regulates proteolysis of ultra-large von Willebrand factor, and reduces platelet aggregation under flow conditions.
18315556 2008 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).
18315546 2008 Prevalence of type 2b 'Malmö/New York' von Willebrand disease in Italy: the role of von Willebrand factor gene conversion.
18263586 2008 Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy.
18194418 2008 Evidence that high von Willebrand factor and low ADAMTS-13 levels independently increase the risk of a non-fatal heart attack.
18173757 2008 Polymorphisms in von Willebrand factor gene promoter influence the glucocorticoid-induced increase in von Willebrand factor: the lesson learned from Cushing syndrome.
18094571 2007 Investigation of von Willebrand factor gene mutations in Korean von Willebrand disease patients.
18064343 2007 A role for von Willebrand factor in Streptococcus sanguis-induced platelet activation.
18045606 2008 Beta3 integrin haplotype influences gene regulation and plasma von Willebrand factor activity.
18042361 2008 Relationship between von Willebrand factor, cholesterol and triglycerides in non-diabetic subjects.
18029348 2008 Toward a confocal subcellular atlas of the human proteome.
17996280 2008 Impaired flow mediated dilatation as evidence of endothelial dysfunction in chronic atrial fibrillation: relationship to plasma von Willebrand factor and soluble E-selectin levels.
17975018 2008 N-linked glycosylation of VWF modulates its interaction with ADAMTS13.
17958741 2007 Requirements for cellular co-trafficking of factor VIII and von Willebrand factor to Weibel-Palade bodies.
17949477 2008 von Willebrand factor: evidence for variable clearance in vivo according to Y/C1584 phenotype and ABO blood group.
17922807 2008 The effect of exercise on von Willebrand factor and ADAMTS-13 in individuals with type 1 and type 2B von Willebrand disease.
17908278 2007 Plasma intercellular adhesion molecule-1 and von Willebrand factor in primary graft dysfunction after lung transplantation.
17901248 2008 Platelet-VWF complexes are preferred substrates of ADAMTS13 under fluid shear stress.
17890957 2007 Haemostatic changes and acquired activated protein C resistance in normal pregnancy.
17883593 2007 Efficiency of von Willebrand factor-mediated targeting of interleukin-8 into Weibel-Palade bodies.
17849054 2007 Effects of toll-like receptor-4 gene polymorphisms on soluble P-selectin and von Willebrand factor levels in hypercholesterolemic patients.
17786534 2008 Correlation between von Willebrand factor antigen, von Willebrand factor ristocetin cofactor activity and factor VIII activity in plasma.
17764538 2007 ADAMTS-13, von Willebrand factor and related parameters in severe sepsis and septic shock.
17723135 2007 Characterization of the interaction between von Willebrand factor and osteoprotegerin.
17721608 2007 Microfluidic reveals generation of platelet-strings on tumor-activated endothelium.
17681836 Molecular study of VWF gene from Mexican Mestizo patients with von Willebrand disease, and the finding of three new mutations.
17650077 2007 Evidence that differential packaging of the major platelet granule proteins von Willebrand factor and fibrinogen can support their differential release.
17622488 2008 Microalbuminuria, von Willebrand factor and fibrinogen levels as markers of the severity in COPD exacerbation.
17619827 2008 Platelet GPIbalpha, GPIV and vWF polymorphisms and fatal pre-hospital MI among middle-aged men.
17598021 2007 Type 2B von Willebrand disease in seven individuals from three different families: phenotypic and genotypic characterization.
17598011 2007 The A/T1381 polymorphism in the A1-domain of von Willebrand factor influences the affinity of von Willebrand factor for platelet glycoprotein Ibalpha.
17597991 2007 Von Willebrand factor: looking back and looking forward.
17493665 2007 The role of von Willebrand factor in thrombus formation.
17490730 2007 Von Willebrand disease - phenotype versus genotype: deficiency versus disease.
17472573 2007 Fluvastatin inhibits regulated secretion of endothelial cell von Willebrand factor in response to diverse secretagogues.
17414220 2007 The presence of active von Willebrand factor under various pathological conditions.
17408410 2007 A mechanism to safeguard platelet adhesion under high shear flow: von Willebrand factor-glycoprotein Ib and integrin alphabeta-collagen interactions make complementary, collagen-type-specific contributions to adhesion.
17408405 2007 Impact of plasma von Willebrand factor levels in the diagnosis of type 1 von Willebrand disease: results from a multicenter European study (MCMDM-1VWD).
17393013 2007 Altered von Willebrand factor subunit proteolysis and multimer processing associated with the Cys2362Phe mutation in the B2 domain.
17393012 2007 Soluble plasma-derived von Willebrand factor assembles to a haemostatically active filamentous network.
17371490 2007 A common 253-kb deletion involving VWF and TMEM16B in German and Italian patients with severe von Willebrand disease type 3.
17296575 2007 The association between the L1565 variant of von Willebrand factor and susceptibility to proteolysis by ADAMTS13.
17275499 The expression of von Willebrand factor, soluble thrombomodulin, and soluble p-selectin during orthotopic liver transplantation.
17190853 2007 The mutational spectrum of type 1 von Willebrand disease: Results from a Canadian cohort study.
17159339 2007 Von Willebrand factor short sequence repeat locus 2 (intron 40) consists of three polymorphic subloci.
17159336 2007 Platelet activation and von Willebrand factor binding to platelets in newborn infants with central venous lines.
17146059 2006 Exosite interactions contribute to tension-induced cleavage of von Willebrand factor by the antithrombotic ADAMTS13 metalloprotease.
17137217 2006 Association of sixty-one non-synonymous polymorphisms in forty-one hypertension candidate genes with blood pressure variation and hypertension.
17121983 2006 Characterization of a core binding site for ADAMTS-13 in the A2 domain of von Willebrand factor.
17119126 2007 Effect of von Willebrand factor Y/C1584 on in vivo protein level and function and interaction with ABO blood group.
17109387 2007 Autosomal recessive von Willebrand disease associated with compound heterozygosity for a novel nonsense mutation (2908 del C) and the missense mutation C2362F: definite evidence for the non-penetrance of the C2362F mutation.
17100655 2006 Von Willebrand factor activates endothelial nitric oxide synthase in blood platelets by a glycoprotein Ib-dependent mechanism.
17098004 2006 Usefulness of von Willebrand factor in cardiac allograft vasculopathy: preliminary experience.
17090649 2007 Variations in glycosylation of von Willebrand factor with O-linked sialylated T antigen are associated with its plasma levels.
17087728 2007 Effect of von Willebrand disease type 2B and type 2M mutations on the susceptibility of von Willebrand factor to ADAMTS-13.
17053057 2007 ADAMTS13 and von Willebrand factor and the risk of myocardial infarction in men.
17052980 2006 Solution structure of human von Willebrand factor studied using small angle neutron scattering.
17010412 2007 Surface-dependent expression in the platelet GPIb binding domain within human von Willebrand factor studied by atomic force microscopy.
16990571 2006 High von Willebrand factor levels increase the risk of first ischemic stroke: influence of ADAMTS13, inflammation, and genetic variability.
16985174 2007 Phenotype and genotype of a cohort of families historically diagnosed with type 1 von Willebrand disease in the European study, Molecular and Clinical Markers for the Diagnosis and Management of Type 1 von Willebrand Disease (MCMDM-1VWD).
16959974 2006 The consensus coding sequences of human breast and colorectal cancers.
16953272 2006 Recombinant factor VIII and factor VIII-von Willebrand factor complex do not present danger signals for human dendritic cells.
16953269 2006 Type 2N von Willebrand disease due to compound heterozygosity for R854Q and a novel R763G mutation at the cleavage site of von Willebrand factor propeptide.
16912226 2006 A single high-affinity binding site for von Willebrand factor in collagen III, identified using synthetic triple-helical peptides.
16899464 2006 Molecular mapping of the chloride-binding site in von Willebrand factor (VWF): energetics and conformational effects on the VWF/ADAMTS-13 interaction.
16894469 2006 R924Q substitution encoded within exon 21 of the von Willebrand factor gene related to mild bleeding phenotype.
16870550 2006 Von Willebrand's disease: a novel mutation, P1824H and the incidence of R1205H defect among families with dominant quantitative von Willebrand factor deficiency.
16839358 2006 Dopamine modulates von Willebrand factor secretion in endothelial cells via D2-D4 receptors.
16835381 2006 Assay of the von Willebrand factor (VWF) propeptide to identify patients with type 1 von Willebrand disease with decreased VWF survival.
16805860 2006 von Willebrand factor A1 domain can adequately substitute for A3 domain in recruitment of flowing platelets to collagen.
16774914 2006 Leucine-rich repeats 2-4 (Leu60-Glu128) of platelet glycoprotein Ibalpha regulate shear-dependent cell adhesion to von Willebrand factor.
16735600 2006 Impact of fibronectin assembly on platelet thrombus formation in response to type I collagen and von Willebrand factor.
16676067 2006 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.
16634765 2006 Physical stress as a model to study variations in ADAMTS-13 activity, von Willebrand factor level and platelet activation.
16581250 2006 p130Cas: a versatile scaffold in signaling networks.
16541075 2006 The finished DNA sequence of human chromosome 12.
16449527 2006 Mechanism of platelet adhesion to von Willebrand factor and microparticle formation under high shear stress.
16435187 2005 Decreased plasma concentration of von Willebrand factor antigen (VWF:Ag) in patients with glycogen storage disease type Ia.
16424652 2006 Von Willebrand factor 1 and factor 2 alleles (intron 40) are suitable markers for carrier detection in von Willebrand disease families in the Indian population.
16420575 2006 The interaction of von Willebrand factor-A1 domain with collagen: mutation G1324S (type 2M von Willebrand disease) impairs the conformational change in A1 domain induced by collagen.
16418330 2006 P-selectin binds to the D'-D3 domains of von Willebrand factor in Weibel-Palade bodies.
16409464 2006 Mutations C1157F and C1234W of von Willebrand factor cause intracellular retention with defective multimerization and secretion.
16373331 2006 Shielding of the A1 domain by the D'D3 domains of von Willebrand factor modulates its interaction with platelet glycoprotein Ib-IX-V.
16344560 2006 Diversification of transcriptional modulation: large-scale identification and characterization of putative alternative promoters of human genes.
16324758 2006 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.
16322474 2006 Impact of mutations in the von Willebrand factor A2 domain on ADAMTS13-dependent proteolysis.
16321553 2006 Genetic mutations in von Willebrand disease identified by DHPLC and DNA sequence analysis.
16320153 2005 Impact of the Thr789Ala variant of the von Willebrand factor levels, on ristocetin co-factor and collagen binding capacity and its association with coronary heart disease in patients with diabetes mellitus type 2.
16314412 2006 Paratope determination of the antithrombotic antibody 82D6A3 based on the crystal structure of its complex with the von Willebrand factor A3-domain.
16286459 2006 Zinc and calcium ions cooperatively modulate ADAMTS13 activity.
16247740 2005 von Willebrand disease R1374C: type 2A or 2M? A challenge to the revised classification. High frequency in the northwest of Spain (Galicia).
16221672 2006 ADAMTS13 substrate recognition of von Willebrand factor A2 domain.
16203734 2005 Enzymatically active ADAMTS13 variants are not inhibited by anti-ADAMTS13 autoantibodies: a novel therapeutic strategy?
16194200 2005 Cysteine-mutations in von Willebrand factor associated with increased clearance.
16141351 2005 Recombinant CUB-1 domain polypeptide inhibits the cleavage of ULVWF strings by ADAMTS13 under flow conditions.
16102037 2005 Cleavage of ultra-large von Willebrand factor by ADAMTS-13 under flow conditions.
16102036 2005 von Willebrand factor: two sides of a coin.
16005629 2005 Integrins and Src: dynamic duo of adhesion signaling.
15975930 2005 The proximal carboxyl-terminal domains of ADAMTS13 determine substrate specificity and are all required for cleavage of von Willebrand factor.
15946221 2005 Endothelial microparticles induce formation of platelet aggregates via a von Willebrand factor/ristocetin dependent pathway, rendering them resistant to dissociation.
15946218 2005 A tripeptide mimetic of von Willebrand factor residues 981-983 enhances platelet adhesion to fibrinogen by signaling through integrin alpha(IIb)beta3.
15933060 2005 Gain of von Willebrand factor-binding function by mutagenesis of a species-conserved residue within the leucine-rich repeat region of platelet glycoprotein Ibalpha.
15886817 2005 Von Willebrand gene tracking by single-tube automated fluorescent analysis of four short tandem repeat polymorphisms.
15886321 2005 Bombay phenotype is associated with reduced plasma-VWF levels and an increased susceptibility to ADAMTS13 proteolysis.
15849757 2005 Von Willebrand factor antigen levels in Behçet disease.
15842375 2005 Evaluation of the von Willebrand factor Y1584C polymorphism as a potential risk factor for bleeding in patients receiving anticoagulant treatment with vitamin K antagonists.
15842374 2005 Theoretical structural explanation for Group I and Group II, type 2A von Willebrand disease mutations.
15842373 2005 Plasma von Willebrand factor and arterial aging.
15831502 2005 Differential kinetics of cell surface loss of von Willebrand factor and its propolypeptide after secretion from Weibel-Palade bodies in living human endothelial cells.
15824096 2005 Binding of ADAMTS13 to von Willebrand factor.
15816602 Plasma von Willebrand factor antigen levels in non-small cell lung cancer patients.
15809291 2005 Role of chloride ions in modulation of the interaction between von Willebrand factor and ADAMTS-13.
15799029 2005 Osteoprotegerin (OPG) is localized to the Weibel-Palade bodies of human vascular endothelial cells and is physically associated with von Willebrand factor.
15764659 2005 Dynamic force spectroscopy of glycoprotein Ib-IX and von Willebrand factor.
15755288 2005 The prevalence of the cysteine1584 variant of von Willebrand factor is increased in type 1 von Willebrand disease: co-segregation with increased susceptibility to ADAMTS13 proteolysis but not clinical phenotype.
15748247 2005 Platelets adhered to endothelial cell-bound ultra-large von Willebrand factor strings support leukocyte tethering and rolling under high shear stress.
15748246 2005 The von Willebrand factor self-association is modulated by a multiple domain interaction.
15735796 2005 The ABO blood group genotype and factor VIII levels as independent risk factors for venous thromboembolism.
15514009 2005 The platelet glycoprotein Ib-von Willebrand factor interaction activates the collagen receptor alpha2beta1 to bind collagen: activation-dependent conformational change of the alpha2-I domain.
15489334 2004 The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC).
15471879 2004 Surface-exposed hemophilic mutations across the factor VIII C2 domain have variable effects on stability and binding activities.
15467896 2004 Increased shear stress- and ristocetin-induced binding of von Willebrand factor to platelets in cord compared with adult plasma.
15459008 2005 Von Willebrand factor accelerates platelet adhesion and thrombus formation on a collagen surface in platelet-reduced blood under flow conditions.
15382239 2004 Experimental indication for the existence of multiple Trp rotamers in von Willebrand Factor A3 domain.
15377475 2004 A new candidate mutation, G1629R, in a patient with type 2A von Willebrand's disease: basic mechanisms and clinical implications.
15331450 2005 Re-establishment of VWF-dependent Weibel-Palade bodies in VWD endothelial cells.
15322948 2004 Molecular modeling of the von Willebrand factor A2 Domain and the effects of associated type 2A von Willebrand disease mutations.
15257287 2004 Rab3D and annexin A2 play a role in regulated secretion of vWF, but not tPA, from endothelial cells.
15249683 2004 Binding of platelet glycoprotein Ibalpha to von Willebrand factor domain A1 stimulates the cleavage of the adjacent domain A2 by ADAMTS13.
15226181 2004 A novel type 2A von Willebrand factor mutation located at the last nucleotide of exon 26 (3538G>A) causes skipping of 2 nonadjacent exons.
15219197 2004 A novel type 2A (Group II) von Willebrand disease mutation (L1503Q) associated with loss of the highest molecular weight von Willebrand factor multimers.
15203711 2004 GPIb potentiates GPVI-induced responses in human platelets.
15182579 2004 [Study of C1423T polymorphism of the von Willebrand factor-cleaving protease gene in Chinese Han population].
15150074 2004 Histone H1-like protein participates in endothelial cell-specific activation of the von Willebrand factor promoter.
15102023 2004 Von Willebrand factor present in fibrillar collagen enhances platelet adhesion to collagen and collagen-induced platelet aggregation.
15087444 2004 ATP augments von Willebrand factor-dependent shear-induced platelet aggregation through Ca2+-calmodulin and myosin light chain kinase activation.
15079089 2004 Crystal structure of the von Willebrand factor A domain of human capillary morphogenesis protein 2: an anthrax toxin receptor.
15039442 2004 Crystal structure of the wild-type von Willebrand factor A1-glycoprotein Ibalpha complex reveals conformation differences with a complex bearing von Willebrand disease mutations.
15029268 2004 Platelet and von Willebrand factor interactions at the vessel wall.
15005754 2004 von Willebrand's disease and psychotic disorders: co-segregation and genetic associations.
14995987 2004 Dimerization and multimerization defects of von Willebrand factor due to mutated cysteine residues.
14760718 2004 Screening for N-glycosylated proteins by liquid chromatography mass spectrometry.
14755371 2004 C1272S: a new candidate mutation in type 2A von Willebrand disease that disrupts the disulfide loop responsible for the interaction of VWF with platelet GP Ib-IX.
14727255 2004 Cleavage of von Willebrand factor by ADAMTS-13 on endothelial cells.
14727254 2004 von Willebrand factor, ADAMTS-13, and thrombotic thrombocytopenic purpura.
14718574 2004 The human plasma proteome: a nonredundant list developed by combination of four separate sources.
14717981 2004 Fibrin polymerization is crucial for thrombin generation in platelet-rich plasma in a VWF-GPIb-dependent process, defective in Bernard-Soulier syndrome.
14717980 2004 von Willebrand factor binding to platelet glycoprotein Ib-IX-V stimulates the assembly of an alpha-actinin-based signaling complex.
14717782 2004 Genetic variability of von Willebrand factor and risk of coronary heart disease: the Rotterdam Study.
14691575 2004 A role for p38 MAP kinase in platelet activation by von Willebrand factor.
14630802 2004 P-selectin anchors newly released ultralarge von Willebrand factor multimers to the endothelial cell surface.
14604963 2004 von Willebrand factor C1C2 domain is involved in platelet adhesion to polymerized fibrin at high shear rate.
14525793 2004 An amino acid polymorphism in von Willebrand factor correlates with increased susceptibility to proteolysis by ADAMTS13.
14517489 2003 Factor VIII and von Willebrand factor changes during normal pregnancy and puerperium.
14512308 2004 VWF73, a region from D1596 to R1668 of von Willebrand factor, provides a minimal substrate for ADAMTS-13.
14507115 2003 Hypoxia and altered platelet behavior influence von Willebrand factor multimeric composition in secondary pulmonary hypertension.
12958606 2003 Anti-heavy-chain monoclonal antibodies directed to the acidic regions of the factor VIII molecule inhibit the binding of factor VIII to phospholipids and von Willebrand factor.
12883448 2003 Von Willebrand factor in type 1 diabetes: its production and coronary artery calcification.
12871509 2003 von Willebrand factor (VWF)-dependent human platelet activation: porcine VWF utilizes different transmembrane signaling pathways than does thrombin to activate platelets, but both require protein phosphatase function.
12871466 2003 von Willebrand factor stimulates thrombin-induced exposure of procoagulant phospholipids on the surface of fibrin-adherent platelets.
12871266 2003 Von Willebrand factor, platelets and endothelial cell interactions.
12860973 2003 Critical roles for the COOH-terminal NITY and RGT sequences of the integrin beta3 cytoplasmic domain in inside-out and outside-in signaling.
12851529 2003 Control of von Willebrand factor multimer size by a fibronectin-related substance.
12815111 2003 The relationship of von Willebrand factor binding to activated platelets from healthy neonates and adults.
12792699 2003 Association studies between -1185A/G von Willebrand factor gene polymorphism and coronary artery disease.
12791651 2003 Analysis of intracellular storage and regulated secretion of 3 von Willebrand disease-causing variants of von Willebrand factor.
12775718 2003 ADAMTS-13 metalloprotease interacts with the endothelial cell-derived ultra-large von Willebrand factor.
12737944 Molecular defects in type 3 von Willebrand disease: updated results from 40 multiethnic patients.
12729795 2003 Von Willebrand factor targets IL-8 to Weibel-Palade bodies in an endothelial cell line.
12719775 2003 Contributions of Asn2198, Met2199, and Phe2200 in the factor VIII C2 domain to cofactor activity, phospholipid-binding, and von Willebrand factor-binding.
12676191 2002 Shear-induced von Willebrand factor-mediated platelet surface translocation of the CD40 ligand.
12668663 2003 Intercellular calcium communication regulates platelet aggregation and thrombus growth.
12665801 2003 Exploring proteomes and analyzing protein processing by mass spectrometric identification of sorted N-terminal peptides.
12649144 2003 Founder von Willebrand factor haplotype associated with type 1 von Willebrand disease.
12646253 2003 Platelet aggregation by membrane-expressed A1 domains of von Willebrand Factor is dependent on residues Asp 560 and Gly 561.
12624629 2003 Genome-wide linkage analysis of von Willebrand factor plasma levels: results from the GAIT project.
12582178 2003 Mapping the collagen-binding site in the von Willebrand factor-A3 domain.
12579041 2003 Von Willebrand factor.
12543870 2003 Activation of pp125FAK by type 2B recombinant von Willebrand factor binding to platelet GPIb at a high shear rate occurs independently of alpha IIb beta 3 engagement.
12511565 2003 The NFY transcription factor inhibits von Willebrand factor promoter activation in non-endothelial cells through recruitment of histone deacetylases.
12477932 2002 Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences.
12456504 2003 Aspects of hydrodynamic shear regulating shear-induced platelet activation and self-association of von Willebrand factor in suspension.
12447349 2003 Collagen-binding mode of vWF-A3 domain determined by a transferred cross-saturation experiment.
12445472 2002 Ca2+ -regulated secretion of tissue-type plasminogen activator and von Willebrand factor in human endothelial cells.
12406074 2002 Factor VIII deficiency not induced by FVIII gene mutation in a female first cousin of two brothers with haemophilia A.
12393736 2003 Interaction between von Willebrand factor and glycoprotein Ib activates Src kinase in human platelets: role of phosphoinositide 3-kinase.
12393698 2003 An Arg760Cys mutation in the consensus sequence of the von Willebrand factor propeptide cleavage site is responsible for a new von Willebrand disease variant.
12393671 2003 Mural thrombus generation in type 2A and 2B von Willebrand disease under flow conditions.
12381897 Polymorphisms in the von Willebrand factor gene are not associated with proliferative retinopathy in non-insulin-dependent diabetes mellitus.
12362242 2002 The mucin-like macroglycopeptide region of glycoprotein Ibalpha is required for cell adhesion to immobilized von Willebrand factor (VWF) under flow but not for static VWF binding.
12361948 2002 Regulation of glycoprotein Ib-IX-von Willebrand factor interaction by cAMP-dependent protein kinase-mediated phosphorylation at Ser 166 of glycoprotein Ib(beta).
12215337 2002 Expression of von Willebrand's factor, CD34, CD31, and vascular endothelial growth factor in uterine leiomyomas.
12183630 2002 Structures of glycoprotein Ibalpha and its complex with von Willebrand factor A1 domain.
12163004 2002 Control of von Willebrand factor multimer size and implications for disease.
12091053 2002 Analysis of the -1185A/G von Willebrand factor (VWF) gene polymorphism in two Brazilian ethnic groups and its effect on the plasma VWF levels.
12087105 2002 Crystal structure of the platelet glycoprotein Ib(alpha) N-terminal domain reveals an unmasking mechanism for receptor activation.
12083486 2002 Factor VIIa induced release of von Willebrand factor from human umbilical vein endothelial cells by a tyrosine kinase dependent pathway.
12082590 2002 The contribution of genetic factors to thrombotic and bleeding outcomes in coronary patients randomised to IIb/IIIa antagonists.
12070018 2002 Reconstitution of adhesive properties of human platelets in liposomes carrying both recombinant glycoproteins Ia/IIa and Ib alpha under flow conditions: specific synergy of receptor-ligand interactions.
12036879 2002 Endothelial von Willebrand factor recruits platelets to atherosclerosis-prone sites in response to hypercholesterolemia.
12036871 2002 Site-directed mutagenesis of platelet glycoprotein Ib alpha demonstrating residues involved in the sulfation of tyrosines 276, 278, and 279.
12006654 2002 Selective and signal-dependent recruitment of membrane proteins to secretory granules formed by heterologously expressed von Willebrand factor.
11992244 2002 Recombinant von Willebrand factor-insight into structure and function through infusion studies in animals with severe von Willebrand disease.
11992236 2002 The role of von Willebrand factor in platelet function.
11943773 2002 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.
11914659 2002 Increased soluble intercellular adhesion molecule-1, breast cancer and the acute phase response.
11864703 2002 Increased levels of endothelial haemostatic markers in patients with coronary heart disease.
11859851 2002 The factor VIII/von Willebrand factor ratio discriminates between reduced synthesis and increased clearance of von Willebrand factor.
11776313 2001 Thrombin-mediated in vitro processing of pro-von Willebrand factor.
11755948 2001 The Sma I polymorphism in the von Willebrand factor gene associated with acute ischemic stroke.
11736957 2001 The -1185 A/G and -1051 G/A dimorphisms in the von Willebrand factor gene promoter and risk of myocardial infarction.
11722428 2001 High factor VIII antigen levels increase the risk of venous thrombosis but are not associated with polymorphisms in the von Willebrand factor and factor VIII gene.
11698279 2001 Type 1 von Willebrand disease mutation Cys1149Arg causes intracellular retention and degradation of heterodimers: a possible general mechanism for dominant mutations of oligomeric proteins.
11583318 2001 Ser968Thr mutation within the A3 domain of von Willebrand factor (VWF) in two related patients leads to a defective binding of VWF to collagen.
11236861 2000 Von Willebrand factor gene polymorphisms in three Brazilian ethnic groups.
11056094 2000 Angiotensin II receptor subtypes in the skeletal muscle vasculature of patients with severe congestive heart failure.
10961880 2000 von Willebrand factor storage and multimerization: 2 independent intracellular processes.
10887121 2000 Phosphoinositide 3-kinase forms a complex with platelet membrane glycoprotein Ib-IX-V complex and 14-3-3zeta.
10887119 2000 A novel von Willebrand disease-causing mutation (Arg273Trp) in the von Willebrand factor propeptide that results in defective multimerization and secretion.
10880054 2000 Vasopressin-induced von Willebrand factor secretion from endothelial cells involves V2 receptors and cAMP.
10831592 2000 Localization of disulfide bonds in the cystine knot domain of human von Willebrand factor.
10764791 2000 Mapping the glycoprotein Ib-binding site in the von willebrand factor A1 domain.
10737800 2000 Shotgun sequencing of the human transcriptome with ORF expressed sequence tags.
10607717 2000 Evidence of increased angiogenesis in patients with acute myeloid leukemia.
10480607 1999 Expression of alpha-1 proteinase inhibitor in human islet microvascular endothelial cells.
9759493 1998 Biochemistry and genetics of von Willebrand factor.
9570915 1998 A simple immunomagnetic protocol for the selective isolation and long-term culture of human dermal microvascular endothelial cells.
9553097 1998 Crystal structure of the von Willebrand Factor A1 domain and implications for the binding of platelet glycoprotein Ib.
9407109 1997 The complete cDNA sequence and structural polymorphism of the polypeptide chain of porcine submaxillary mucin.
9373253 1997 Activation of human platelets by the membrane-expressed A1 domain of von Willebrand factor.
9351824 1997 Regulation of the pp72syk protein tyrosine kinase by platelet integrin alpha IIb beta 3.
9331419 1997 Crystal structure of the A3 domain of human von Willebrand factor: implications for collagen binding.
9312128 1997 The von willebrand factor A3 domain does not contain a metal ion-dependent adhesion site motif.
9218428 1997 The acidic region of the factor VIII light chain and the C2 domain together form the high affinity binding site for von willebrand factor.
9215757 1997 Crystal structure of NMC-4 fab anti-von Willebrand factor A1 domain.
9079671 1997 Propolypeptide of von Willebrand factor is a novel ligand for very late antigen-4 integrin.
8874190 1996 Shear-dependent changes in the three-dimensional structure of human von Willebrand factor.
8839833 1996 Dominant type 1 von Willebrand disease caused by mutated cysteine residues in the D3 domain of von Willebrand factor.
8622978 1996 Defective dimerization of von Willebrand factor subunits due to a Cys-> Arg mutation in type IID von Willebrand disease.
8590955 1996 Characterization of adhesion receptors on cultured microvascular endothelial cells derived from the retroorbital connective tissue of patients with Grave's ophthalmopathy.
8565074 1996 Initiation of platelet adhesion by arrest onto fibrinogen or translocation on von Willebrand factor.
8547152 1995 Effects of different amino-acid substitutions in the leucine 694-proline 708 segment of recombinant von Willebrand factor.
8486782 1993 von Willebrand factor mutation enhancing interaction with platelets in patients with normal multimeric structure.
8435341 1993 Substitution of cysteine for phenylalanine 751 in mature von Willebrand factor is a novel candidate mutation in a family with type IIA von Willebrand disease.
8376405 1993 Type IIB mutation His-505-->Asp implicates a new segment in the control of von Willebrand factor binding to platelet glycoprotein Ib.
8348943 1993 Two new candidate mutations in type IIA von Willebrand's disease (Arg834-->Gly, Gly846-->Arg) and one polymorphism (Tyr821-->Cys) in the A2 region of the von Willebrand factor.
8338947 1993 Identification of three candidate mutations causing type IIA von Willebrand disease using a rapid, nonradioactive, allele-specific hybridization method.
8318738 1993 Conserved linkage of neurotrophin-3 and von Willebrand factor on mouse chromosome 6.
8123844 1994 Characterization of Leu777Pro and Ile865Thr type IIA von Willebrand disease mutations.
8123843 1994 Leu 697-->Val mutation in mature von Willebrand factor is responsible for type IIB von Willebrand disease.
8088787 1994 Characterization of the von Willebrand factor gene (VWF) in von Willebrand disease type III patients from 24 families of Swedish and Finnish origin.
8011991 1994 Investigation of type IIC von Willebrand disease.
7989040 1994 Genetic heterogeneity of severe von Willebrand disease type III in the German population.
7789955 1995 Identification of a candidate missense mutation in a family with von Willebrand disease type IIC.
7756647 1995 The affinity and stoichiometry of binding of human factor VIII to von Willebrand factor.
7734373 1995 A novel candidate mutation (Arg611-->His) in type I 'platelet discordant' von Willebrand's disease with desmopressin-induced thrombocytopenia.
7721887 1995 Identification of three tyrosine residues of glycoprotein Ib alpha with distinct roles in von Willebrand factor and alpha-thrombin binding.
7620154 1995 Identification of two mutations (Arg611Cys and Arg611His) in the A1 loop of von Willebrand factor (vWF) responsible for type 2 von Willebrand disease with decreased platelet-dependent function of vWF.
7505120 1993 Solid-phase von Willebrand factor contains a conformationally active RGD motif that mediates endothelial cell adhesion through the alpha v beta 3 receptor.
6754744 1982 Immunolocalization of von Willebrand protein in Weibel-Palade bodies of human endothelial cells.
6336654 1983 Studies with a murine monoclonal antibody that abolishes ristocetin-induced binding of von Willebrand factor to platelets: additional evidence in support of GPIb as a platelet receptor for von Willebrand factor.
3875078 1985 Construction of cDNA coding for human von Willebrand factor using antibody probes for colony-screening and mapping of the chromosomal gene.
3874428 1985 Human von Willebrand factor (vWF): isolation of complementary DNA (cDNA) clones and chromosomal localization.
3873280 1985 Molecular cloning of cDNA for human von Willebrand factor: authentication by a new method.
3872140 1985 Factor VIII/von Willebrand factor in subendothelium mediates platelet adhesion.
3524673 1986 Amino acid sequence of human von Willebrand factor.
3502076 1987 Identification of disulfide-bridged substructures within human von Willebrand factor.
3496594 1987 Molecular cloning of the human gene for von Willebrand factor and identification of the transcription initiation site.
3495266 1987 Evolution of human von Willebrand factor: cDNA sequence polymorphisms, repeated domains, and relationship to von Willebrand antigen II.
3490481 1986 Isolation and characterization of a collagen binding domain in human von Willebrand factor.
3489923 1986 Nucleotide sequence of pre-pro-von Willebrand factor cDNA.
3489483 1986 von Willebrand factor, now cloned.
3488775 1986 von Willebrand's disease characterized by increased ristocetin sensitivity and the presence of all von Willebrand factor multimers in plasma.
3488076 1986 cDNA sequences for human von Willebrand factor reveal five types of repeated domains and five possible protein sequence polymorphisms.
3121636 1987 The relationship of N-linked glycosylation and heavy chain-binding protein association with the secretion of glycoproteins.
3089784 1986 Primary structure of a new tetraantennary glycan of the N-acetyllactosaminic type isolated from human factor VIII/von Willebrand factor.
3087627 1986 Inducible secretion of large, biologically potent von Willebrand factor multimers.
3082891 1986 Initial glycosylation and acidic pH in the Golgi apparatus are required for multimerization of von Willebrand factor.
3033024 1987 Gene deletions correlate with the development of alloantibodies in von Willebrand disease.
3019665 1986 Full-length von Willebrand factor (vWF) cDNA encodes a highly repetitive protein considerably larger than the mature vWF subunit.
2864688 1985 Cloning and characterization of two cDNAs coding for human von Willebrand factor.
2839553 1988 Evidence that the primary binding site of von Willebrand factor that mediates platelet adhesion on subendothelium is not collagen.
2828057 1988 The human von Willebrand factor gene. Structure of the 5' region.
2786201 1989 Molecular basis of human von Willebrand disease: analysis of platelet von Willebrand factor mRNA.