JAX
Mouse Strain Datasheet - 003795
B6.129S2-Vwftm1Wgr/J
Also Known As: vWf KO
These Vwf knock-out mice exhibit defects in hemostasis characterized by prolonged bleeding times.
Donating Investigator
Denisa D. Wagner, Center for Blood Research
Genetic overview
| Genetic Background | Generation |
|---|---|
|
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Vwftm1Wgr
| Allele Type | Gene Symbol | Gene Name |
|---|---|---|
| Targeted (Null/Knockout) | Vwf | Von Willebrand factor |
Detailed Description
Mice that are homozygous null for the Vwf gene are viable, fertile, normal in size and do not display any gross physical or behavioral abnormalities. No Vwf protein product is detected in platelets, plasma, heart or lung endothelium. Null mice exhibit defects in hemostasis characterized by prolonged bleeding times in all mice with spontaneous bleeding events observed in ten percent of neonates. Intravital microscopic analysis indicates a complete absence of thrombus formation following vascular injury. Observed levels of factor VIII are reduced to twenty percent of that seen in wild type mice. Although heterozygous mice carrying a single null allele exhibit no defects in hemostasis, they do have reduced levels of factor VIII (57 percent that of wildtype) making them a suitable model for type 1 von Willebrand disease. Characteristics displayed by homozygous null animals qualify them as an appropriate model for severe (type 3) von Willebrand disease.
Development
A targeting vector containing a neomycin resistance gene driven by a mouse phosphoglycerate kinase promoter flanked by two herpes simplex virus thymidine kinase cassettes was used to disrupt exons four and five of the Vwf gene. The construct was introduced into 129S2/SvPas-derived D3 embryonic stem (ES) cells by electroporation. Correctly targeted ES cells were injected into C57BL/6J blastocysts. The resulting chimeric male animals were backcrossed to C57BL/6J females.
Control Suggestions
- Wild-type from the colony
- 000664 C57BL/6J
Additional Information
Vwftm1Wgr
Allele Symbol: Vwftm1Wgr
| Allele Name | targeted mutation 1, Denisa D Wagner |
|---|---|
| Allele Type | Targeted (Null/Knockout) |
| Allele Synonym(s) | VWF KO; vWf- |
| Gene Symbol and Name | Vwf, Von Willebrand factor |
| Gene Synonym(s) | 6820430P06Rik; 6820430P06Rik; AI551257; B130011O06Rik; B130011O06Rik; F8VWF; RIKEN cDNA 6820430P06 gene; RIKEN cDNA B130011O06 gene; VWD; expressed sequence AI551257 |
| Strain of Origin | 129S2/SvPas |
| Chromosome | 6 |
| Molecular Note | A neomycin cassette was inserted into intron 5. Northern blot analysis revealed that no normal transcripts were present in homozygous mice, and immunofluorescence experiments demonstrated that the protein was not present in blood smears of homozygous mice. |
| Mutations Made By | Cecile Denis, Center for Blood Research |
Disease Terms
Research Areas By Genotype
This mouse can be used to support research in many areas including:
Genotype: Vwftm1Wgr related
- Hematological Research
- Clotting Defects
Mammalian Phenotype Terms by Genotype
Genotype: Vwftm1Wgr/Vwftm1Wgr
B6.129S2-Vwftm1Wgr
immune system phenotype
- increased susceptibility to bacterial infection
- following treatment with complete Freund's adjuvant (CFA) and pertussis toxin, blood-brain barrier permeability is increased compared to in similarly treated wild-type mice (MGI Ref ID J:140334)
- pertussis toxin-treated mice exhibit longer persistence of HA sensitization than similarly treated wild-type mice (MGI Ref ID J:140334)
- increased susceptibility to experimental autoimmune encephalomyelitis
- however, mice exhibit normal experimental autoimmune encephalomyelitis in the spinal cord and encephalitogenic T cell response (MGI Ref ID J:140334)
- mice treated with MOG35-55, complete Freund's adjuvant (CFA), and pertussis toxin exhibit more severe clinical symptoms (lesions scores, demyelination, suppuration, mononuclear cellular infiltration, blood brain barrier permeability) in the brain and enhanced duration of the acute-early phase compared with similarly treated wild-type micenced duration of the acute-early phase compared with similarly treated wild-type mice (MGI Ref ID J:140334)
cardiovascular system phenotype
- abnormal blood-brain barrier function
- following treatment with MOG35-55, complete Freund's adjuvant (CFA), and pertussis toxin (PTX) or only CFA and PTX, blood-brain barrier permeability is increased compared to in similarly treated wild-type mice (MGI Ref ID J:140334)
homeostasis/metabolism phenotype
- abnormal blood coagulation
- following FeCl3 injury, mice exhibit a increased time to clot formation and fail to completely occlude vessels unlike similarly treated wild-type mice (MGI Ref ID J:133469)
nervous system phenotype
- abnormal blood-brain barrier function
- following treatment with MOG35-55, complete Freund's adjuvant (CFA), and pertussis toxin (PTX) or only CFA and PTX, blood-brain barrier permeability is increased compared to in similarly treated wild-type mice (MGI Ref ID J:140334)
The following phenotype information is associated with a similar, but not exact match to this JAX® Mice strain
Genotype: Vwftm1Wgr/Vwf+
involves: 129S2/SvPas * C57BL/6J
homeostasis/metabolism phenotype
- abnormal blood coagulation
- in heterozygotes, the activity level of coagulation FVIII was reduced to 57% of wild-type level (MGI Ref ID J:49083)
Genotype: Vwftm1Wgr/Vwftm1Wgr
involves: 129S2/SvPas
homeostasis/metabolism phenotype
- decreased myocardial infarction size
- following middle cerebral artery occlusion (MGI Ref ID J:148295)
- decreased susceptibility to ischemic brain injury
- following middle cerebral artery occlusion, mice exhibit smaller infarct size and reduced neurological deficits compared with similarly treated wild-type mice (MGI Ref ID J:148295)
cardiovascular system phenotype
- decreased myocardial infarction size
- following middle cerebral artery occlusion (MGI Ref ID J:148295)
nervous system phenotype
- decreased susceptibility to ischemic brain injury
- following middle cerebral artery occlusion, mice exhibit smaller infarct size and reduced neurological deficits compared with similarly treated wild-type mice (MGI Ref ID J:148295)
Genotype: Vwftm1Wgr/Vwftm1Wgr
involves: 129S2/SvPas * C57BL/6J
cardiovascular system phenotype
- internal hemorrhage
- after the neonatal period, no obvious spontaneous bleeding was observed, and females survived pregnancy and delivery of normal size litters (MGI Ref ID J:49083)
- in some cases, bleeding was massive and proved fatal (MGI Ref ID J:49083)
- ~10% of newborn homozygotes displayed spontaneous intra-abdominal bleeding (MGI Ref ID J:49083)
homeostasis/metabolism phenotype
- abnormal blood coagulation
- after FeCl3 treatment, platelet interaction with the vessel wall is delayed and platelet deposition is 5-fold less than in similarly treated wild-type mice (MGI Ref ID J:63750)
- after FeCl3 treatment, thrombi fail to occlude blood vessels unlike in similarly treated wild-type mice (MGI Ref ID J:63750)
- consistent with a reduction in FVIII, the activated partial thromboplastin time (aPTT) was also prolonged while prothrombin time was not changed (MGI Ref ID J:49083)
- however, platelet, red cell and white cell counts, hematocrit, and hemoglobin were all within normal range (MGI Ref ID J:49083)
- in homozygotes, the activity level of coagulation FVIII was reduced to 20% of wild-type level (MGI Ref ID J:49083)
- abnormal platelet physiology
- at 10 min after ferric chloride-induced injury, most mutant arterioles (66.6%) showed very few, if any, platelet interactions with the vessel wall, whereas all of wild-type arterioles exhibited either complete occlusion (25%) or numerous platelet interactions with the vessel wall, including formation of thrombiions with the vessel wall, including formation of thrombi (MGI Ref ID J:49083)
- increased bleeding time
- following amputation of a tail segment, homozygotes exhibited a significantly prolonged bleeding time relative to wild-type mice (499 33.4 sec versus 69.7 5.2 sec, respectively) (MGI Ref ID J:49083)
- in addition, 2 anesthetized animals that were not cauterized were never able to control their bleeding (MGI Ref ID J:49083)
- only 5 of 21 homozygotes managed to control their blood loss without cauterization (MGI Ref ID J:49083)
digestive/alimentary phenotype
- melena
- 7.2% of adult homozygotes displayed the presence of fecal occult blood (MGI Ref ID J:49083)
hematopoietic system phenotype
- abnormal platelet physiology
- at 10 min after ferric chloride-induced injury, most mutant arterioles (66.6%) showed very few, if any, platelet interactions with the vessel wall, whereas all of wild-type arterioles exhibited either complete occlusion (25%) or numerous platelet interactions with the vessel wall, including formation of thrombiions with the vessel wall, including formation of thrombi (MGI Ref ID J:49083)
References
Additional References
- Denis CV; Kwack K; Saffaripour S; Maganti S; Andre P; Schaub RG; Wagner DD. 2001. Interleukin 11 significantly increases plasma von Willebrand factor and factor VIII in wild type and von Willebrand disease mouse models. Blood 97(2):465-72. PubMed: 11154224MGI: J:66963
- Ni H; Denis CV; Subbarao S; Degen JL; Sato TN; Hynes RO; Wagner DD. 2000. Persistence of platelet thrombus formation in arterioles of mice lacking both von Willebrand factor and fibrinogen. J Clin Invest 106(3):385-92. PubMed: 10930441MGI: J:63750
Additional - Vwftm1Wgr related
- Andre P; Denis CV; Ware J; Saffaripour S; Hynes RO; Ruggeri ZM; Wagner DD. 2000. Platelets adhere to and translocate on von willebrand factor presented by endothelium in stimulated veins Blood 96(10):3322-8. PubMed: 11071623MGI: J:65812
- Barr JD; Chauhan AK; Schaeffer GV; Hansen JK; Motto DG. 2013. Red blood cells mediate the onset of thrombosis in the ferric chloride murine model. Blood 121(18):3733-41. PubMed: 23343833MGI: J:197474
- Brill A; Fuchs TA; Chauhan AK; Yang JJ; De Meyer SF; Kollnberger M; Wakefield TW; Lammle B; Massberg S; Wagner DD. 2011. von Willebrand factor-mediated platelet adhesion is critical for deep vein thrombosis in mouse models. Blood 117(4):1400-7. PubMed: 20959603MGI: J:168416
- Casari C; Berrou E; Lebret M; Adam F; Kauskot A; Bobe R; Desconclois C; Fressinaud E; Christophe OD; Lenting PJ; Rosa JP; Denis CV; Bryckaert M. 2013. von Willebrand factor mutation promotes thrombocytopathy by inhibiting integrin alphaIIbbeta3. J Clin Invest 123(12):5071-81. PubMed: 24270421MGI: J:207833
- Casari C; Du V; Wu YP; Kauskot A; de Groot PG; Christophe OD; Denis CV; de Laat B; Lenting PJ. 2013. Accelerated uptake of VWF/platelet complexes in macrophages contributes to VWD type 2B-associated thrombocytopenia. Blood 122(16):2893-902. PubMed: 23945153MGI: J:203262
- Chauhan AK; Kisucka J; Brill A; Walsh MT; Scheiflinger F; Wagner DD. 2008. ADAMTS13: a new link between thrombosis and inflammation. J Exp Med 205(9):2065-74. PubMed: 18695007MGI: J:142125
- Chauhan AK; Kisucka J; Lamb CB; Bergmeier W; Wagner DD. 2007. von Willebrand factor and factor VIII are independently required to form stable occlusive thrombi in injured veins. Blood 109(6):2424-9. PubMed: 17119108MGI: J:145354
- Chauhan AK; Walsh MT; Zhu G; Ginsburg D; Wagner DD; Motto DG. 2008. The combined roles of ADAMTS13 and VWF in murine models of TTP, endotoxemia, and thrombosis. Blood 111(7):3452-7. PubMed: 18083848MGI: J:133469
- Chen J; Zhou H; Diacovo A; Zheng XL; Emsley J; Diacovo TG. 2014. Exploiting the kinetic interplay between GPIbalpha-VWF binding interfaces to regulate hemostasis and thrombosis. Blood 124(25):3799-807. PubMed: 25293780MGI: J:220792
- Chion A; O'Sullivan JM; Drakeford C; Bergsson G; Dalton N; Aguila S; Ward S; Fallon PG; Brophy TM; Preston RJ; Brady L; Sheils O; Laffan M; McKinnon TA; O'Donnell JS. 2016. N-linked glycans within the A2 domain of von Willebrand factor modulate macrophage-mediated clearance. Blood 128(15):1959-1968. PubMed: 27554083MGI: J:237234
- Cosemans JM; Schols SE; Stefanini L; de Witt S; Feijge MA; Hamulyak K; Deckmyn H; Bergmeier W; Heemskerk JW. 2011. Key role of glycoprotein Ib/V/IX and von Willebrand factor in platelet activation-dependent fibrin formation at low shear flow. Blood 117(2):651-60. PubMed: 21037087MGI: J:168396
- De Meyer SF; Schwarz T; Deckmyn H; Denis CV; Nieswandt B; Stoll G; Vanhoorelbeke K; Kleinschnitz C. 2010. Binding of von Willebrand factor to collagen and glycoprotein Ibalpha, but not to glycoprotein IIb/IIIa, contributes to ischemic stroke in mice--brief report. Arterioscler Thromb Vasc Biol 30(10):1949-51. PubMed: 20616311MGI: J:182111
- De Meyer SF; Vandeputte N; Pareyn I; Petrus I; Lenting PJ; Chuah MK; VandenDriessche T; Deckmyn H; Vanhoorelbeke K. 2008. Restoration of plasma von Willebrand factor deficiency is sufficient to correct thrombus formation after gene therapy for severe von Willebrand disease. Arterioscler Thromb Vasc Biol 28(9):1621-6. PubMed: 18556568MGI: J:159807
- Denis C; Methia N; Frenette PS; Rayburn H; Ullman-Cullere M ; Hynes RO ; Wagner DD. 1998. A mouse model of severe von Willebrand disease: defects in hemostasis and thrombosis. Proc Natl Acad Sci U S A 95(16):9524-9. PubMed: 9689113MGI: J:49083
- Denis CV; Kwack K; Saffaripour S; Maganti S; Andre P; Schaub RG; Wagner DD. 2001. Interleukin 11 significantly increases plasma von Willebrand factor and factor VIII in wild type and von Willebrand disease mouse models. Blood 97(2):465-72. PubMed: 11154224MGI: J:66963
- Dole VS; Bergmeier W; Mitchell HA; Eichenberger SC; Wagner DD. 2005. Activated platelets induce Weibel-Palade-body secretion and leukocyte rolling in vivo: role of P-selectin. Blood 106(7):2334-9. PubMed: 15956287MGI: J:119376
- Dubois C; Panicot-Dubois L; Gainor JF; Furie BC; Furie B. 2007. Thrombin-initiated platelet activation in vivo is vWF independent during thrombus formation in a laser injury model. J Clin Invest 117(4):953-60. PubMed: 17380206MGI: J:121278
- Dunne E; Spring CM; Reheman A; Jin W; Berndt MC; Newman DK; Newman PJ; Ni H; Kenny D. 2012. Cadherin 6 has a functional role in platelet aggregation and thrombus formation. Arterioscler Thromb Vasc Biol 32(7):1724-31. PubMed: 22539596MGI: J:201502
- Frederix K; Chauhan AK; Kisucka J; Zhao BQ; Hoff EI; Spronk HM; Ten Cate H; Wagner DD. 2007. Platelet adhesion receptors do not modulate infarct volume after a photochemically induced stroke in mice. Brain Res 1185:239-45. PubMed: 17996853MGI: J:130124
- Gandhi C; Motto DG; Jensen M; Lentz SR; Chauhan AK. 2012. ADAMTS13 deficiency exacerbates VWF-dependent acute myocardial ischemia/reperfusion injury in mice. Blood 120(26):5224-30. PubMed: 22983446MGI: J:192128
- Goerge T; Ho-Tin-Noe B; Carbo C; Benarafa C; Remold-O'Donnell E; Zhao BQ; Cifuni SM; Wagner DD. 2008. Inflammation induces hemorrhage in thrombocytopenia. Blood 111(10):4958-64. PubMed: 18256319MGI: J:135316
- Hillgruber C; Steingraber AK; Poppelmann B; Denis CV; Ware J; Vestweber D; Nieswandt B; Schneider SW; Goerge T. 2014. Blocking von Willebrand factor for treatment of cutaneous inflammation. J Invest Dermatol 134(1):77-86. PubMed: 23812299MGI: J:205720
- Hoffmeister KM; Felbinger TW; Falet H; Denis CV; Bergmeier W; Mayadas TN; von Andrian UH; Wagner DD; Stossel TP; Hartwig JH. 2003. The clearance mechanism of chilled blood platelets. Cell 112(1):87-97. PubMed: 12526796MGI: J:107707
- Holmback K; Danton MJ; Suh TT; Daugherty CC; Degen JL. 1996. Impaired platelet aggregation and sustained bleeding in mice lacking the fibrinogen motif bound by integrin alpha IIb beta 3. EMBO J 15(21):5760-71. PubMed: 8918453MGI: J:36873
- Kleinschnitz C; De Meyer SF; Schwarz T; Austinat M; Vanhoorelbeke K; Nieswandt B; Deckmyn H; Stoll G. 2009. Deficiency of von Willebrand factor protects mice from ischemic stroke. Blood 113(15):3600-3. PubMed: 19182208MGI: J:148295
- Le Behot A; Gauberti M; Martinez De Lizarrondo S; Montagne A; Lemarchand E; Repesse Y; Guillou S; Denis CV; Maubert E; Orset C; Vivien D. 2014. GpIbalpha-VWF blockade restores vessel patency by dissolving platelet aggregates formed under very high shear rate in mice. Blood 123(21):3354-63. PubMed: 24553181MGI: J:210753
- Legendre P; Navarrete AM; Rayes J; Casari C; Boisseau P; Ternisien C; Caron C; Fressinaud E; Goudemand J; Veyradier A; Denis CV; Lenting PJ; Christophe OD. 2013. Mutations in the A3 domain of von Willebrand factor inducing combined qualitative and quantitative defects in the protein. Blood 121(11):2135-43. PubMed: 23335371MGI: J:195864
- Marx I; Christophe OD; Lenting PJ; Rupin A; Vallez MO; Verbeuren TJ; Denis CV. 2008. Altered thrombus formation in von Willebrand factor-deficient mice expressing von Willebrand factor variants with defective binding to collagen or GPIIbIIIa. Blood 112(3):603-9. PubMed: 18487513MGI: J:138441
- Marx I; Lenting PJ; Adler T; Pendu R; Christophe OD; Denis CV. 2008. Correction of bleeding symptoms in von Willebrand factor-deficient mice by liver-expressed von Willebrand factor mutants. Arterioscler Thromb Vasc Biol 28(3):419-24. PubMed: 18187670MGI: J:159817
- Meeks SL; Cox CL; Healey JF; Parker ET; Doshi BS; Gangadharan B; Barrow RT; Lollar P. 2012. A major determinant of the immunogenicity of factor VIII in a murine model is independent of its procoagulant function. Blood 120(12):2512-20. PubMed: 22855607MGI: J:191310
- Mei B; Pan C; Jiang H; Tjandra H; Strauss J; Chen Y; Liu T; Zhang X; Severs J; Newgren J; Chen J; Gu JM; Subramanyam B; Fournel MA; Pierce GF; Murphy JE. 2010. Rational design of a fully active, long-acting PEGylated factor VIII for hemophilia A treatment. Blood 116(2):270-9. PubMed: 20194895MGI: J:162833
- Methia N; Andre P; Denis CV; Economopoulos M; Wagner DD. 2001. Localized reduction of atherosclerosis in von Willebrand factor-deficient mice. Blood 98(5):1424-8. PubMed: 11520791MGI: J:106677
- Morioka Y; Casari C; Wohner N; Cho S; Kurata S; Kitano A; Christophe OD; Lenting PJ; Li R; Denis CV; Prevost N. 2014. Expression of a structurally constrained von Willebrand factor variant triggers acute thrombotic thrombocytopenic purpura in mice. Blood 123(21):3344-53. PubMed: 24713928MGI: J:213135
- Navarrete AM; Casari C; Legendre P; Marx I; Hu JR; Lenting PJ; Christophe OD; Denis CV. 2012. A murine model to characterize the antithrombotic effect of molecules targeting human von Willebrand factor. Blood 120(13):2723-32. PubMed: 22915646MGI: J:191809
- Ni H; Denis CV; Subbarao S; Degen JL; Sato TN; Hynes RO; Wagner DD. 2000. Persistence of platelet thrombus formation in arterioles of mice lacking both von Willebrand factor and fibrinogen. J Clin Invest 106(3):385-92. PubMed: 10930441MGI: J:63750
- Noubade R; del Rio R; McElvany B; Zachary JF; Millward JM; Wagner DD; Offner H; Blankenhorn EP; Teuscher C. 2008. von-Willebrand factor influences blood brain barrier permeability and brain inflammation in experimental allergic encephalomyelitis. Am J Pathol 173(3):892-900. PubMed: 18688020MGI: J:140334
- O'Regan N; Gegenbauer K; O'Sullivan JM; Maleki S; Brophy TM; Dalton N; Chion A; Fallon PG; Grau GE; Budde U; Smith OP; Craig AG; Preston RJ; O'Donnell JS. 2016. A novel role for von Willebrand factor in the pathogenesis of experimental cerebral malaria. Blood 127(9):1192-201. PubMed: 26511133MGI: J:231351
- Othman M; Labelle A; Mazzetti I; Elbatarny HS; Lillicrap D. 2007. Adenovirus-induced thrombocytopenia: the role of von Willebrand factor and P-selectin in mediating accelerated platelet clearance. Blood 109(7):2832-9. PubMed: 17148587MGI: J:145348
- Patel KN; Soubra SH; Bellera RV; Dong JF; McMullen CA; Burns AR; Rumbaut RE. 2008. Differential role of von Willebrand factor and P-selectin on microvascular thrombosis in endotoxemia. Arterioscler Thromb Vasc Biol 28(12):2225-30. PubMed: 18802014MGI: J:159795
- Petri B; Broermann A; Li H; Khandoga AG; Zarbock A; Krombach F; Goerge T; Schneider SW; Jones C; Nieswandt B; Wild MK; Vestweber D. 2010. von Willebrand factor promotes leukocyte extravasation. Blood 116(22):4712-9. PubMed: 20716766MGI: J:166636
- Poirault-Chassac S; Nguyen KA; Pietrzyk A; Casari C; Veyradier A; Denis CV; Baruch D. 2013. Terminal platelet production is regulated by von Willebrand factor. PLoS One 8(5):e63810. PubMed: 23737952MGI: J:200787
- Prakash P; Kulkarni PP; Chauhan AK. 2015. Thrombospondin 1 requires von Willebrand factor to modulate arterial thrombosis in mice. Blood 125(2):399-406. PubMed: 25343959MGI: J:220781
- Pruss CM; Golder M; Bryant A; Hegadorn CA; Burnett E; Laverty K; Sponagle K; Dhala A; Notley C; Haberichter S; Lillicrap D. 2011. Pathologic mechanisms of type 1 VWD mutations R1205H and Y1584C through in vitro and in vivo mouse models. Blood 117(16):4358-66. PubMed: 21346256MGI: J:172829
- Rayes J; Hollestelle MJ; Legendre P; Marx I; de Groot PG; Christophe OD; Lenting PJ; Denis CV. 2010. Mutation and ADAMTS13-dependent modulation of disease severity in a mouse model for von Willebrand disease type 2B. Blood 115(23):4870-7. PubMed: 20200350MGI: J:161564
- Reheman A; Yang H; Zhu G; Jin W; He F; Spring CM; Bai X; Gross PL; Freedman J; Ni H. 2009. Plasma fibronectin depletion enhances platelet aggregation and thrombus formation in mice lacking fibrinogen and von Willebrand factor. Blood 113(8):1809-17. PubMed: 19036705MGI: J:145550
- Saint-Lu N; Oortwijn BD; Pegon JN; Odouard S; Christophe OD; de Groot PG; Denis CV; Lenting PJ. 2012. Identification of galectin-1 and galectin-3 as novel partners for von Willebrand factor. Arterioscler Thromb Vasc Biol 32(4):894-901. PubMed: 22267483MGI: J:195956
- Schaff M; Tang C; Maurer E; Bourdon C; Receveur N; Eckly A; Hechler B; Arnold C; de Arcangelis A; Nieswandt B; Denis CV; Lefebvre O; Georges-Labouesse E; Gachet C; Lanza F; Mangin PH. 2013. Integrin alpha6beta1 is the main receptor for vascular laminins and plays a role in platelet adhesion, activation, and arterial thrombosis. Circulation 128(5):541-52. PubMed: 23797810MGI: J:209493
- Scheppke L; Murphy EA; Zarpellon A; Hofmann JJ; Merkulova A; Shields DJ; Weis SM; Byzova TV; Ruggeri ZM; Iruela-Arispe ML; Cheresh DA. 2012. Notch promotes vascular maturation by inducing integrin-mediated smooth muscle cell adhesion to the endothelial basement membrane. Blood 119(9):2149-58. PubMed: 22134168MGI: J:182467
- Schwarz HP; Lenting PJ; Binder B; Mihaly J; Denis C; Dorner F; Turecek PL. 2000. Involvement of low-density lipoprotein receptor-related protein (LRP) in the clearance of factor VIII in von Willebrand factor-deficient mice. Blood 95(5):1703-8. PubMed: 10688827MGI: J:60764
- Shao L; Sun Y; Zhang Z; Feng W; Gao Y; Cai Z; Wang ZZ; Look AT; Wu WS. 2010. Deletion of proapoptotic Puma selectively protects hematopoietic stem and progenitor cells against high-dose radiation. Blood 115(23):4707-14. PubMed: 20360471MGI: J:161544
- Shi Q; Fahs SA; Kuether EL; Cooley BC; Weiler H; Montgomery RR. 2010. Targeting FVIII expression to endothelial cells regenerates a releasable pool of FVIII and restores hemostasis in a mouse model of hemophilia A. Blood 116(16):3049-57. PubMed: 20606161MGI: J:165877
- Shi Q; Wilcox DA; Fahs SA; Weiler H; Wells CW; Cooley BC; Desai D; Morateck PA; Gorski J; Montgomery RR. 2006. Factor VIII ectopically targeted to platelets is therapeutic in hemophilia A with high-titer inhibitory antibodies. J Clin Invest 116(7):1974-82. PubMed: 16823491MGI: J:111741
- Shida Y; Rydz N; Stegner D; Brown C; Mewburn J; Sponagle K; Danisment O; Crawford B; Vidal B; Hegadorn CA; Pruss CM; Nieswandt B; Lillicrap D. 2014. Analysis of the role of von Willebrand factor, platelet glycoprotein VI-, and alpha2beta1-mediated collagen binding in thrombus formation. Blood 124(11):1799-807. PubMed: 25051961MGI: J:214616
- Starke RD; Ferraro F; Paschalaki KE; Dryden NH; McKinnon TA; Sutton RE; Payne EM; Haskard DO; Hughes AD; Cutler DF; Laffan MA; Randi AM. 2011. Endothelial von Willebrand factor regulates angiogenesis. Blood 117(3):1071-80. PubMed: 21048155MGI: J:177805
- Stennicke HR; Kjalke M; Karpf DM; Balling KW; Johansen PB; Elm T; Ovlisen K; Moller F; Holmberg HL; Gudme CN; Persson E; Hilden I; Pelzer H; Rahbek-Nielsen H; Jespersgaard C; Bogsnes A; Pedersen AA; Kristensen AK; Peschke B; Kappers W; Rode F; Thim L; Tranholm M; Ezban M; Olsen EH; Bjorn SE. 2013. A novel B-domain O-glycoPEGylated FVIII (N8-GP) demonstrates full efficacy and prolonged effect in hemophilic mice models. Blood 121(11):2108-16. PubMed: 23335368MGI: J:195866
- Verhenne S; Denorme F; Libbrecht S; Vandenbulcke A; Pareyn I; Deckmyn H; Lambrecht A; Nieswandt B; Kleinschnitz C; Vanhoorelbeke K; De Meyer SF. 2015. Platelet-derived VWF is not essential for normal thrombosis and hemostasis but fosters ischemic stroke injury in mice. Blood 126(14):1715-22. PubMed: 26209660MGI: J:230256
- Wang Y; Reheman A; Spring CM; Kalantari J; Marshall AH; Wolberg AS; Gross PL; Weitz JI; Rand ML; Mosher DF; Freedman J; Ni H. 2014. Plasma fibronectin supports hemostasis and regulates thrombosis. J Clin Invest 124(10):4281-93. PubMed: 25180602MGI: J:217640
- Yang H; Lang S; Zhai Z; Li L; Kahr WH; Chen P; Brkic J; Spring CM; Flick MJ; Degen JL; Freedman J; Ni H. 2009. Fibrinogen is required for maintenance of platelet intracellular and cell-surface P-selectin expression. Blood 114(2):425-36. PubMed: 19332769MGI: J:150774
- Yang H; Reheman A; Chen P; Zhu G; Hynes RO; Freedman J; Wagner DD; Ni H. 2006. Fibrinogen and von Willebrand factor-independent platelet aggregation in vitro and in vivo. J Thromb Haemost 4(10):2230-7. PubMed: 16824188MGI: J:129287
- Yarovoi H; Nurden AT; Montgomery RR; Nurden P; Poncz M. 2005. Intracellular interaction of von Willebrand factor and factor VIII depends on cellular context: lessons from platelet-expressed factor VIII. Blood 105(12):4674-6. PubMed: 15731176MGI: J:107451
- Zhao BQ; Chauhan AK; Canault M; Patten IS; Yang JJ; Dockal M; Scheiflinger F; Wagner DD. 2009. von Willebrand factor-cleaving protease ADAMTS13 reduces ischemic brain injury in experimental stroke. Blood 114(15):3329-34. PubMed: 19687510MGI: J:153541
- van Schooten CJ; Shahbazi S; Groot E; Oortwijn BD; van den Berg HM; Denis CV; Lenting PJ. 2008. Macrophages contribute to the cellular uptake of von Willebrand factor and factor VIII in vivo. Blood 112(5):1704-12. PubMed: 18559674MGI: J:138724