Gene Symbol: Vwf
Description: Von Willebrand factor
Alias: 6820430P06Rik, AI551257, B130011O06Rik, C630030D09, F8VWF, VWD, von Willebrand factor, Von Willebrand factor homolog, vwf
Species: mouse
Products:     Vwf

Top Publications

  1. Methia N, Andre P, Denis C, Economopoulos M, Wagner D. Localized reduction of atherosclerosis in von Willebrand factor-deficient mice. Blood. 2001;98:1424-8 pubmed
    To examine the role of the platelet adhesion molecule von Willebrand factor (vWf) in atherogenesis, vWf-deficient mice (vWf-/-) were bred with mice lacking the low-density lipoprotein receptor (LDLR-/-) on a C57BL/6J background...
  2. Shiojiri N, Niwa T, Sugiyama Y, Koike T. Preferential expression of connexin37 and connexin40 in the endothelium of the portal veins during mouse liver development. Cell Tissue Res. 2006;324:547-52 pubmed
    ..The differentiation of each blood vessel in the hepatic vascular system may occur in early developmental stages, soon after hepatic primordium formation. ..
  3. Mohlke K, Nichols W, Westrick R, Novak E, Cooney K, Swank R, et al. A novel modifier gene for plasma von Willebrand factor level maps to distal mouse chromosome 11. Proc Natl Acad Sci U S A. 1996;93:15352-7 pubmed
    Type 1 von Willebrand disease (VWD), characterized by reduced levels of plasma von Willebrand factor (VWF), is the most common inherited bleeding disorder in humans...
  4. Rayes J, Hollestelle M, Legendre P, Marx I, de Groot P, Christophe O, et al. Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B. Blood. 2010;115:4870-7 pubmed publisher
    Von Willebrand disease (VWD)-type 2B originates from a gain-of-function mutation in von Willebrand factor (VWF), resulting in enhanced platelet binding...
  5. Andre P, Denis C, Ware J, Saffaripour S, Hynes R, Ruggeri Z, et al. Platelets adhere to and translocate on von Willebrand factor presented by endothelium in stimulated veins. Blood. 2000;96:3322-8 pubmed
    ..s, and then adhesion progressively decreased. This adhesion process was dependent on von Willebrand factor (vWF) and independent of P-selectin...
  6. Lemmerhirt H, Shavit J, Levy G, Cole S, Long J, Ginsburg D. Enhanced VWF biosynthesis and elevated plasma VWF due to a natural variant in the murine Vwf gene. Blood. 2006;108:3061-7 pubmed
    Both genetic and environmental influences contribute to the wide variation in plasma von Willebrand factor (VWF) levels observed in humans...
  7. Matsui T, Kanai Azuma M, Hara K, Matoba S, Hiramatsu R, Kawakami H, et al. Redundant roles of Sox17 and Sox18 in postnatal angiogenesis in mice. J Cell Sci. 2006;119:3513-26 pubmed
    ..Therefore, our findings indicate that Sox17 and Sox18, and possibly all three SoxF genes, are cooperatively involved in mammalian vascular development. ..
  8. Chauhan A, Walsh M, Zhu G, Ginsburg D, Wagner D, Motto D. The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis. Blood. 2008;111:3452-7 pubmed
    Ultralarge von Willebrand factor (UL-VWF) multimers are thought to play a central role in pathogenesis of the disease thrombotic thrombocytopenic purpura (TTP); however, experimental evidence in support of this hypothesis has been ..
  9. Nichols W, Cooney K, Mohlke K, Ballew J, Yang A, Bruck M, et al. von Willebrand disease in the RIIIS/J mouse is caused by a defect outside of the von Willebrand factor gene. Blood. 1994;83:3225-31 pubmed
    ..Murine vWD is characterized by a prolonged bleeding time, normal von Willebrand factor (vWF) multimer distribution, autosomal dominant inheritance, and proportionately decreased plasma vWF ..

More Information

Publications101 found, 100 shown here

  1. Lenting P, Westein E, Terraube V, Ribba A, Huizinga E, Meyer D, et al. An experimental model to study the in vivo survival of von Willebrand factor. Basic aspects and application to the R1205H mutation. J Biol Chem. 2004;279:12102-9 pubmed
    To explore the molecular basis of von Willebrand factor (VWF) clearance, an experimental model employing VWF-deficient mice was developed...
  2. Pergolizzi R, Jin G, Chan D, Pierre L, Bussel J, Ferris B, et al. Correction of a murine model of von Willebrand disease by gene transfer. Blood. 2006;108:862-9 pubmed
    ..S. population, is caused by defects in the expression and processing of von Willebrand factor (VWF), a blood glycoprotein required for normal hemostasis that mediates the adhesion of platelets to ..
  3. Marx I, Christophe O, Lenting P, Rupin A, Vallez M, Verbeuren T, et al. Altered thrombus formation in von Willebrand factor-deficient mice expressing von Willebrand factor variants with defective binding to collagen or GPIIbIIIa. Blood. 2008;112:603-9 pubmed publisher
    The role of von Willebrand factor (VWF) in thrombosis involves its binding to a number of ligands...
  4. Denis C, Methia N, Frenette P, Rayburn H, Ullman Cullere M, Hynes R, et al. A mouse model of severe von Willebrand disease: defects in hemostasis and thrombosis. Proc Natl Acad Sci U S A. 1998;95:9524-9 pubmed
    b>von Willebrand factor (vWf) deficiency causes severe von Willebrand disease in humans. We generated a mouse model for this disease by using gene targeting. vWf-deficient mice appeared normal at birth; they were viable and fertile...
  5. Dole V, Bergmeier W, Mitchell H, Eichenberger S, Wagner D. Activated platelets induce Weibel-Palade-body secretion and leukocyte rolling in vivo: role of P-selectin. Blood. 2005;106:2334-9 pubmed
    ..Endothelial P-selectin is stored with von Willebrand factor (VWF) in Weibel-Palade bodies...
  6. Chauhan A, Kisucka J, Lamb C, Bergmeier W, Wagner D. von Willebrand factor and factor VIII are independently required to form stable occlusive thrombi in injured veins. Blood. 2007;109:2424-9 pubmed
    b>von Willebrand factor (VWF) protects factor VIII (FVIII) from proteolysis and mediates the initial contact of platelets with the injured vessel wall, thus playing an important role in hemostasis and thrombosis...
  7. Lemmerhirt H, Broman K, Shavit J, Ginsburg D. Genetic regulation of plasma von Willebrand factor levels: quantitative trait loci analysis in a mouse model. J Thromb Haemost. 2007;5:329-35 pubmed
    The genetic factors responsible for the wide variation in plasma von Willebrand factor (VWF) levels observed among individuals are largely unknown, although these genes are also likely to contribute to variability in the severity of von ..
  8. Marx I, Lenting P, Adler T, Pendu R, Christophe O, Denis C. Correction of bleeding symptoms in von Willebrand factor-deficient mice by liver-expressed von Willebrand factor mutants. Arterioscler Thromb Vasc Biol. 2008;28:419-24 pubmed publisher
    b>von Willebrand Factor (vWF) structure-function relationship has been studied only in vitro...
  9. Rathore V, Wang D, Newman D, Newman P. Phospholipase Cgamma2 contributes to stable thrombus formation on VWF. FEBS Lett. 2004;573:26-30 pubmed
    ..we examined the ability of wild-type and PLCgamma2- deficient murine platelets to spread on immobilized von Willebrand factor (VWF) under static conditions, and to attach to and form thrombi on VWF under conditions of arterial shear...
  10. Mehedint M, Craciunescu C, Zeisel S. Maternal dietary choline deficiency alters angiogenesis in fetal mouse hippocampus. Proc Natl Acad Sci U S A. 2010;107:12834-9 pubmed publisher
    ..01). We conclude that maternal dietary choline intake alters angiogenesis in the developing fetal hippocampus. ..
  11. Ida Yonemochi H, Nakajima M, Saku T. Heparanase, heparan sulfate and perlecan distribution along with the vascular penetration during stellate reticulum retraction in the mouse enamel organ. Arch Oral Biol. 2010;55:778-87 pubmed publisher
  12. Kaur S, Leszczynska K, Abraham S, Scarcia M, Hiltbrunner S, Marshall C, et al. RhoJ/TCL regulates endothelial motility and tube formation and modulates actomyosin contractility and focal adhesion numbers. Arterioscler Thromb Vasc Biol. 2011;31:657-64 pubmed publisher
  13. Holmbäck K, Danton M, Suh T, Daugherty C, Degen J. Impaired platelet aggregation and sustained bleeding in mice lacking the fibrinogen motif bound by integrin alpha IIb beta 3. EMBO J. 1996;15:5760-71 pubmed
    ..Despite the preservation of clotting function, gamma delta 5/gamma delta 5 mice were unable to control blood loss following a surgical challenge and occasionally developed fatal neonatal bleeding events. ..
  14. Yoshioka M, Yuasa S, Matsumura K, Kimura K, Shiomi T, Kimura N, et al. Chondromodulin-I maintains cardiac valvular function by preventing angiogenesis. Nat Med. 2006;12:1151-9 pubmed
    ..These findings provide evidence that chondromodulin-I has a pivotal role in maintaining valvular normal function by preventing angiogenesis that may lead to VHD. ..
  15. Tanjore H, Zeisberg E, Gerami Naini B, Kalluri R. Beta1 integrin expression on endothelial cells is required for angiogenesis but not for vasculogenesis. Dev Dyn. 2008;237:75-82 pubmed
    ..These results indicate that beta1 integrin expression on vascular endothelial cells is crucial for embryonic angiogenesis but dispensable for vasculogenesis. ..
  16. Chung M, Popova T, Jorgensen S, Dong L, Chandhoke V, Bailey C, et al. Degradation of circulating von Willebrand factor and its regulator ADAMTS13 implicates secreted Bacillus anthracis metalloproteases in anthrax consumptive coagulopathy. J Biol Chem. 2008;283:9531-42 pubmed publisher
    ..In this study we hypothesized that anthrax infection modulates the activity of von Willebrand factor (VWF) and its endogenous regulator ADAMTS13, which play important roles in hemostasis and thrombosis, ..
  17. Stoller J, Degenhardt K, Huang L, Zhou D, Lu M, Epstein J. Cre reporter mouse expressing a nuclear localized fusion of GFP and beta-galactosidase reveals new derivatives of Pax3-expressing precursors. Genesis. 2008;46:200-4 pubmed publisher
    ..Thus, we report a previously unrecognized subset of venous endothelial cells derived from Pax3 expressing precursors. ..
  18. Popova T, Millis B, Bailey C, Popov S. Platelets, inflammatory cells, von Willebrand factor, syndecan-1, fibrin, fibronectin, and bacteria co-localize in the liver thrombi of Bacillus anthracis-infected mice. Microb Pathog. 2012;52:1-9 pubmed publisher
    ..for syndecan-1 (SDC-1), fibronectin, and were surrounded by fibrin polymers, GPIIb-positive platelets, von Willebrand Factor (vWF), CD45-positive leukocytes, and massive amount of shed SDC-1...
  19. Dunne E, Spring C, Reheman A, Jin W, Berndt M, Newman D, et al. Cadherin 6 has a functional role in platelet aggregation and thrombus formation. Arterioscler Thromb Vasc Biol. 2012;32:1724-31 pubmed publisher
    ..Because platelet aggregation occurs in fibrinogen and von Willebrand factor double-deficient mice, we investigated whether cadherin 6 is an alternative ligand for the integrin ?(IIb)?..
  20. Khan M, Motto D, Lentz S, Chauhan A. ADAMTS13 reduces VWF-mediated acute inflammation following focal cerebral ischemia in mice. J Thromb Haemost. 2012;10:1665-71 pubmed publisher
    ADAMTS13 cleaves hyperactive ultra-large von Willebrand factor (ULVWF) multimers into smaller and less active forms...
  21. Epaud R, Aubey F, Xu J, Chaker Z, Clemessy M, Dautin A, et al. Knockout of insulin-like growth factor-1 receptor impairs distal lung morphogenesis. PLoS ONE. 2012;7:e48071 pubmed publisher
    ..Immunohistochemistry using pro-SP-C, NKX2-1, CD31 and vWF as markers revealed a delay in cell differentiation and arrest in the canalicular stage of prenatal respiratory ..
  22. Stennicke H, Kjalke M, Karpf D, Balling K, Johansen P, Elm T, et al. A novel B-domain O-glycoPEGylated FVIII (N8-GP) demonstrates full efficacy and prolonged effect in hemophilic mice models. Blood. 2013;121:2108-16 pubmed publisher
    ..Binding to von Willebrand factor (VWF) was maintained for all conjugates...
  23. Janel N, Schibler L, Oustry A, Kerbiriou Nabias D, Cribiu E, Vaiman D. The localization of the von Willebrand factor gene on cattle, sheep and goat chromosomes illustrates karyotype evolution in mammals. Mamm Genome. 1996;7:633-4 pubmed
  24. Colvin J, White A, Pratt S, Ornitz D. Lung hypoplasia and neonatal death in Fgf9-null mice identify this gene as an essential regulator of lung mesenchyme. Development. 2001;128:2095-106 pubmed
    ..We suggest a model whereby FGF9 signaling from the epithelium and reciprocal FGF10 signaling from the mesenchyme coordinately regulate epithelial airway branching and organ size during lung embryogenesis. ..
  25. De Maeyer B, De Meyer S, Feys H, Pareyn I, Vandeputte N, Deckmyn H, et al. The distal carboxyterminal domains of murine ADAMTS13 influence proteolysis of platelet-decorated VWF strings in vivo. J Thromb Haemost. 2010;8:2305-12 pubmed publisher
    The multidomain metalloprotease ADAMTS13 regulates the size of von Willebrand factor (VWF) multimers upon their release from endothelial cells...
  26. Pappelbaum K, Gorzelanny C, Grässle S, Suckau J, Laschke M, Bischoff M, et al. Ultralarge von Willebrand factor fibers mediate luminal Staphylococcus aureus adhesion to an intact endothelial cell layer under shear stress. Circulation. 2013;128:50-9 pubmed publisher
    ..bacteria under high-shear-rate conditions revealed 95% attachment of the S aureus by ultralarge von Willebrand factor (ULVWF)...
  27. Chislock E, Ring C, Pendergast A. Abl kinases are required for vascular function, Tie2 expression, and angiopoietin-1-mediated survival. Proc Natl Acad Sci U S A. 2013;110:12432-7 pubmed publisher
    ..These findings reveal a critical requirement for the Abl kinases in vascular development and function, which may have important implications for the clinical use of Abl kinase inhibitors. ..
  28. Schatteman G, Motley S, Effmann E, Bowen Pope D. Platelet-derived growth factor receptor alpha subunit deleted Patch mouse exhibits severe cardiovascular dysmorphogenesis. Teratology. 1995;51:351-66 pubmed
  29. Subramanian S, Nadal Ginard B. Early expression of the different isoforms of the myocyte enhancer factor-2 (MEF2) protein in myogenic as well as non-myogenic cell lineages during mouse embryogenesis. Mech Dev. 1996;57:103-12 pubmed
    ..Their presence in non-muscle cells further suggests they could also play a role in the determination of other mesodermal derivatives, including cells of the vasculature. ..
  30. Shi Q, Fahs S, Kuether E, Cooley B, Weiler H, Montgomery R. Targeting FVIII expression to endothelial cells regenerates a releasable pool of FVIII and restores hemostasis in a mouse model of hemophilia A. Blood. 2010;116:3049-57 pubmed publisher
    ..cells produces an intracellular pool of FVIII that can be mobilized together with its carrier protein, von Willebrand factor (VWF), by agonists...
  31. Brill A, Fuchs T, Chauhan A, Yang J, De Meyer S, Köllnberger M, et al. von Willebrand factor-mediated platelet adhesion is critical for deep vein thrombosis in mouse models. Blood. 2011;117:1400-7 pubmed publisher
    ..b>von Willebrand factor (VWF)-deficient mice were protected from thrombosis induced by complete (stasis) or partial (stenosis) ..
  32. Leineweber S, Schönig S, Seeger K. Insight into interactions of the von-Willebrand-factor-A-like domain 2 with the FNIII-like domain 9 of collagen VII by NMR and SPR. FEBS Lett. 2011;585:1748-52 pubmed publisher
    ..Based on these assignments a secondary structure prediction was performed showing a properly folded protein. An interaction of mvWFA2 with its neighboring domain mFNIII-9 was characterized with NMR spectroscopy and SPR. ..
  33. Pruss C, Golder M, Bryant A, Hegadorn C, Haberichter S, Lillicrap D. Use of a mouse model to elucidate the phenotypic effects of the von Willebrand factor cleavage mutants, Y1605A/M1606A and R1597W. J Thromb Haemost. 2012;10:940-50 pubmed publisher
    b>von Willebrand Factor (VWF) is tightly regulated by the metalloproteinase ADAMTS13, which cleaves VWF to reduce VWF multimer size and binding affinity for collagen and platelets...
  34. Chen W, Druzak S, Wang Y, Josephson C, Hoffmeister K, Ware J, et al. Refrigeration-Induced Binding of von Willebrand Factor Facilitates Fast Clearance of Refrigerated Platelets. Arterioscler Thromb Vasc Biol. 2017;37:2271-2279 pubmed publisher
    ..In this study, the role of von Willebrand factor (VWF) in the clearance of refrigerated platelets is addressed...
  35. Ni H, Denis C, Subbarao S, Degen J, Sato T, Hynes R, et al. Persistence of platelet thrombus formation in arterioles of mice lacking both von Willebrand factor and fibrinogen. J Clin Invest. 2000;106:385-92 pubmed
    ..With this model, we evaluated thrombus growth in mice lacking von Willebrand factor (vWF) and fibrinogen (Fg), the two key ligands known to mediate platelet adhesion and aggregation...
  36. Chauhan A, Kisucka J, Brill A, Walsh M, Scheiflinger F, Wagner D. ADAMTS13: a new link between thrombosis and inflammation. J Exp Med. 2008;205:2065-74 pubmed publisher
    b>von Willebrand factor (VWF) levels are elevated and a disintegrin-like and metalloprotease with thrombospondin type I repeats-13 (ADAMTS13) activity is decreased in both acute and chronic inflammation...
  37. Madeja Z, Yadi H, Apps R, Boulenouar S, Roper S, Gardner L, et al. Paternal MHC expression on mouse trophoblast affects uterine vascularization and fetal growth. Proc Natl Acad Sci U S A. 2011;108:4012-7 pubmed publisher
    ..Thus, maternal-fetal immune interactions are instrumental to optimize reproductive success. This cross-talk has important implications for human disorders of pregnancy, such as preeclampsia and fetal growth restriction. ..
  38. Casari C, Berrou E, Lebret M, Adam F, Kauskot A, Bobe R, et al. von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin ?IIb?3. J Clin Invest. 2013;123:5071-81 pubmed publisher
    von Willebrand disease type 2B (vWD-type 2B) is characterized by gain-of-function mutations in von Willebrand factor (vWF) that enhance its binding to the glycoprotein Ib-IX-V complex on platelets...
  39. Le Béhot A, Gauberti M, Martinez de Lizarrondo S, Montagne A, Lemarchand E, Repesse Y, et al. GpIb?-VWF blockade restores vessel patency by dissolving platelet aggregates formed under very high shear rate in mice. Blood. 2014;123:3354-63 pubmed publisher
    ..are GpIIb/IIIa-dependent, our findings reveal that closure of the arterial lumen is mediated by GpIb?-von Willebrand Factor (VWF) interactions...
  40. Zhu X, Cao Y, Wei L, Cai P, Xu H, Luo H, et al. von Willebrand factor contributes to poor outcome in a mouse model of intracerebral haemorrhage. Sci Rep. 2016;6:35901 pubmed publisher
    ..b>von Willebrand factor (VWF) has recently been demonstrated to promote inflammation processes...
  41. Yang H, Reheman A, Chen P, Zhu G, Hynes R, Freedman J, et al. Fibrinogen and von Willebrand factor-independent platelet aggregation in vitro and in vivo. J Thromb Haemost. 2006;4:2230-7 pubmed
    ..demonstrated formation of occlusive thrombi in Fg-deficient mice and in mice doubly deficient for Fg and von Willebrand factor (Fg/VWF(-/-))...
  42. Srinivasan R, Oliver G. Prox1 dosage controls the number of lymphatic endothelial cell progenitors and the formation of the lymphovenous valves. Genes Dev. 2011;25:2187-97 pubmed publisher
    ..This is the first report describing the molecular mechanism controlling lymphovenous communication. ..
  43. Oommen S, Francois M, Kawasaki M, Murrell M, Kawasaki K, Porntaveetus T, et al. Cytoplasmic plaque formation in hemidesmosome development is dependent on SoxF transcription factor function. PLoS ONE. 2012;7:e43857 pubmed publisher
    ..Our data suggest that SOXF transcription factors play a role in regulating formation of cytoplasmic plaque protein assembly, and that disrupted SOXF function results in epidermolysis bullosa-like skin phenotypes. ..
  44. Savchenko A, Borissoff J, Martinod K, De Meyer S, Gallant M, Erpenbeck L, et al. VWF-mediated leukocyte recruitment with chromatin decondensation by PAD4 increases myocardial ischemia/reperfusion injury in mice. Blood. 2014;123:141-8 pubmed publisher
    ..Recombinant human ADAMTS13 (rhADAMTS13), cleaving von Willebrand factor (VWF), reduces leukocyte recruitment in mice...
  45. Wohner N, Legendre P, Casari C, Christophe O, Lenting P, Denis C. Shear stress-independent binding of von Willebrand factor-type 2B mutants p.R1306Q & p.V1316M to LRP1 explains their increased clearance. J Thromb Haemost. 2015;13:815-20 pubmed publisher
    b>von Willebrand factor (VWF) is cleared in a shear stress- and macrophage-dependent manner by LRP1. von Willebrand disease (VWD)-type 2B mutants are endocytosed more efficiently than wild-type (wt)-VWF by macrophages...
  46. Bultmann Mellin I, Dinger K, Debuschewitz C, Loewe K, Melcher Y, Plum M, et al. Role of LTBP4 in alveolarization, angiogenesis, and fibrosis in lungs. Am J Physiol Lung Cell Mol Physiol. 2017;313:L687-L698 pubmed publisher
  47. Oblander S, Zhou Z, Galvez B, Starcher B, Shannon J, Durbeej M, et al. Distinctive functions of membrane type 1 matrix-metalloprotease (MT1-MMP or MMP-14) in lung and submandibular gland development are independent of its role in pro-MMP-2 activation. Dev Biol. 2005;277:255-69 pubmed
  48. Casari C, Du V, Wu Y, Kauskot A, de Groot P, Christophe O, et al. Accelerated uptake of VWF/platelet complexes in macrophages contributes to VWD type 2B-associated thrombocytopenia. Blood. 2013;122:2893-902 pubmed publisher
    Von Willebrand disease (VWD) type 2B is characterized by mutations causing enhanced binding of von Willebrand factor (VWF) to platelets...
  49. Sjoberg A, Seaman W, Bellinger D, Griggs T, Nichols T, Chowdhary B. FISH mapping of the porcine vWF gene to chromosome 5q21 extends synteny homology with human chromosome 12. Hereditas. 1996;124:199-202 pubmed
  50. Velling T, Collo G, Sorokin L, Durbeej M, Zhang H, Gullberg D. Distinct alpha 7A beta 1 and alpha 7B beta 1 integrin expression patterns during mouse development: alpha 7A is restricted to skeletal muscle but alpha 7B is expressed in striated muscle, vasculature, and nervous system. Dev Dyn. 1996;207:355-71 pubmed
    ..We conclude that the alpha 7A and alpha 7B integrin variants are expressed in a developmentally regulated, tissue-specific pattern suggesting different functions for the two splice forms. ..
  51. Rosen E, Chan J, Idusogie E, Clotman F, Vlasuk G, Luther T, et al. Mice lacking factor VII develop normally but suffer fatal perinatal bleeding. Nature. 1997;390:290-4 pubmed
    ..Thus, the TF-/- embryonic lethal and the FVII-/- survival-phenotypes suggest a role for TF during embryogenesis beyond fibrin formation. ..
  52. Arthur H, Ure J, Smith A, Renforth G, Wilson D, Torsney E, et al. Endoglin, an ancillary TGFbeta receptor, is required for extraembryonic angiogenesis and plays a key role in heart development. Dev Biol. 2000;217:42-53 pubmed
    ..We anticipate that heterozygous mice will ultimately serve as a useful disease model for HHT1, as some individuals have dilated and fragile blood vessels similar to vascular malformations seen in HHT patients. ..
  53. Yuan B, Li C, Kimura S, Engelhardt R, Smith B, Minoo P. Inhibition of distal lung morphogenesis in Nkx2.1(-/-) embryos. Dev Dyn. 2000;217:180-90 pubmed
    ..1 independent, while distal lung morphogenesis appears to be strictly dependent on the wild-type activity of Nkx2.1. ..
  54. Iwaki T, Sandoval Cooper M, Paiva M, Kobayashi T, Ploplis V, Castellino F. Fibrinogen stabilizes placental-maternal attachment during embryonic development in the mouse. Am J Pathol. 2002;160:1021-34 pubmed
  55. Petrova T, Karpanen T, Norrmen C, Mellor R, Tamakoshi T, Finegold D, et al. Defective valves and abnormal mural cell recruitment underlie lymphatic vascular failure in lymphedema distichiasis. Nat Med. 2004;10:974-81 pubmed
    ..Our results indicate that an abnormal interaction between the lymphatic endothelial cells and pericytes, as well as valve defects, underlie the pathogenesis of LD. ..
  56. Hou S, Chan L, Chou Y, Su C, Chen X, Shih Y, et al. Ankrd17, an ubiquitously expressed ankyrin factor, is essential for the vascular integrity during embryogenesis. FEBS Lett. 2009;583:2765-71 pubmed publisher
    ..The Ankrd17-deficient mice also provide a new animal model for the analysis of the regulatory pathways of the differentiation of vSMC precursor cells. ..
  57. Rusu L, Andreeva A, Visintine D, Kim K, Vogel S, Stojanovic Terpo A, et al. G protein-dependent basal and evoked endothelial cell vWF secretion. Blood. 2014;123:442-50 pubmed publisher
    b>von Willebrand factor (vWF) secretion by endothelial cells (ECs) is essential for hemostasis and thrombosis; however, the molecular mechanisms are poorly understood...
  58. Prakash P, Kulkarni P, Chauhan A. Thrombospondin 1 requires von Willebrand factor to modulate arterial thrombosis in mice. Blood. 2015;125:399-406 pubmed publisher
    ..a counter receptor to platelet glycoprotein Ibα that supports initial platelet adhesion in absence of von Willebrand factor (VWF)...
  59. Bauer A, Suckau J, Frank K, Desch A, Goertz L, Wagner A, et al. von Willebrand factor fibers promote cancer-associated platelet aggregation in malignant melanoma of mice and humans. Blood. 2015;125:3153-63 pubmed publisher
    ..Although it is known that activation of ECs and the release of the procoagulatory protein von Willebrand factor (VWF) is essential for malignancy, the underlying mechanisms remain poorly understood...
  60. Ahlfeld S, Wang J, Gao Y, Snider P, Conway S. Initial Suppression of Transforming Growth Factor-β Signaling and Loss of TGFBI Causes Early Alveolar Structural Defects Resulting in Bronchopulmonary Dysplasia. Am J Pathol. 2016;186:777-93 pubmed publisher
    ..These studies underline the complex (and often contradictory) role of TGF-β and indicate a need to design studies to associate alterations with initial appearance of phenotypical changes suggestive of bronchopulmonary dysplasia. ..
  61. Coffin J, Harrison J, Schwartz S, Heimark R. Angioblast differentiation and morphogenesis of the vascular endothelium in the mouse embryo. Dev Biol. 1991;148:51-62 pubmed
    Bandeiraea simplicifolia B4 isolectin (BSLB4) and polyclonal antisera against von Willebrand factor (VWF) were used to study the origin of endothelial cells and their organization into blood vessels in the postimplantation mouse embryo...
  62. Aigner S, Ruppert M, Hubbe M, Sammar M, Sthoeger Z, Butcher E, et al. Heat stable antigen (mouse CD24) supports myeloid cell binding to endothelial and platelet P-selectin. Int Immunol. 1995;7:1557-65 pubmed
    ..Our data suggest that HSA, in addition to the recently identified 160 kDa glycoprotein ligand on mouse neutrophils, belongs to a group of monospecific P-selectin ligands on myeloid cells. ..
  63. Denis C, Kwack K, Saffaripour S, Maganti S, Andre P, Schaub R, et al. Interleukin 11 significantly increases plasma von Willebrand factor and factor VIII in wild type and von Willebrand disease mouse models. Blood. 2001;97:465-72 pubmed
    Interleukin (IL)-11 is a cytokine with thrombopoietic activity that has been shown to increase plasma von Willebrand factor (vWf) in preliminary clinical studies...
  64. Shi Q, Wilcox D, Fahs S, Weiler H, Wells C, Cooley B, et al. Factor VIII ectopically targeted to platelets is therapeutic in hemophilia A with high-titer inhibitory antibodies. J Clin Invest. 2006;116:1974-82 pubmed
    ..Our results demonstrate that this approach to the targeted expression of FVIII in platelets has the potential to correct hemophilia A, even in the presence of inhibitory immune responses to infused FVIII. ..
  65. High F, Lu M, Pear W, Loomes K, Kaestner K, Epstein J. Endothelial expression of the Notch ligand Jagged1 is required for vascular smooth muscle development. Proc Natl Acad Sci U S A. 2008;105:1955-9 pubmed publisher
    ..Together, these results imply that the primary role of endothelial Jag1 is to potentiate the development of neighboring vascular smooth muscle. ..
  66. Rawley O, O Sullivan J, Chion A, Keyes S, Lavin M, van Rooijen N, et al. von Willebrand factor arginine 1205 substitution results in accelerated macrophage-dependent clearance in vivo. J Thromb Haemost. 2015;13:821-6 pubmed publisher
    Enhanced von Willebrand factor (VWF) clearance is important in the etiology of type 1 and type 2 von Willebrand disease (VWD). More than 20 different VWF point mutations have already been reported in patients with enhanced clearance...
  67. Miragall F, Krause D, de Vries U, Dermietzel R. Expression of the tight junction protein ZO-1 in the olfactory system: presence of ZO-1 on olfactory sensory neurons and glial cells. J Comp Neurol. 1994;341:433-48 pubmed
    ..Our results provide further evidence that ZO-1 serves functions unrelated to the tight junction complex and indicate molecular heterogeneity of these cell-cell contacts. ..
  68. Othman M, Labelle A, Mazzetti I, Elbatarny H, Lillicrap D. Adenovirus-induced thrombocytopenia: the role of von Willebrand factor and P-selectin in mediating accelerated platelet clearance. Blood. 2007;109:2832-9 pubmed
    ..In this study, we have assessed the influence of von Willebrand Factor (VWF) and P-selectin on the clearance of platelets following adenovirus administration...
  69. Park J, Lee H, Ko K, Park S, Kim J, Choe G, et al. Meteorin regulates angiogenesis at the gliovascular interface. Glia. 2008;56:247-58 pubmed
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    ..transporter-like protein-2 (CTL-2), was reported to interact with a protein of the inner ear carrying 2 von Willebrand factor (VWF) A-domains...
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    One of the major determinants of von Willebrand factor (VWF) plasma levels is ABO blood group status, and individuals with blood group O have ~ 25% lower plasma levels...
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