Strain Name:

B6.129S2-Vwftm1Wgr/J

Stock Number:

003795

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Description

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Strain Information

Type Congenic; Mutant Strain; Targeted Mutation;
Additional information on Genetically Engineered and Mutant Mice.
Visit our online Nomenclature tutorial.
Additional information on Congenic nomenclature.
Specieslaboratory mouse
 
Donating Investigator Denisa D. Wagner,   Center for Blood Research

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 Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Model with phenotypic similarity to human disease where etiologies involve orthologs. Human genes are associated with this disease. Orthologs of those genes appear in the mouse genotype(s).
Von Willebrand Disease, Type 1; VWD1
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Von Willebrand Disease, Type 2; VWD2   (VWF)
Von Willebrand Disease, Type 3; VWD3   (VWF)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

Vwftm1Wgr/Vwftm1Wgr

        B6.129S2-Vwftm1Wgr
  • immune system phenotype
  • increased susceptibility to bacterial infection
    • pertussis toxin-treated mice exhibit longer persistence of HA sensitization than similarly treated wild-type mice   (MGI Ref ID J:140334)
    • 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)
  • increased susceptibility to experimental autoimmune encephalomyelitis
    • 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 mice   (MGI Ref ID J:140334)
    • however, mice exhibit normal experimental autoimmune encephalomyelitis in the spinal cord and encephalitogenic T cell response   (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.

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)

Vwftm1Wgr/Vwftm1Wgr

        involves: 129S2/SvPas * C57BL/6J
  • cardiovascular system phenotype
  • internal hemorrhage
    • ~10% of newborn homozygotes displayed spontaneous intra-abdominal bleeding   (MGI Ref ID J:49083)
    • in some cases, bleeding was massive and proved fatal   (MGI Ref ID J:49083)
    • 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)
  • homeostasis/metabolism phenotype
  • abnormal blood coagulation
    • in homozygotes, the activity level of coagulation FVIII was reduced to 20% of wild-type level   (MGI Ref ID J:49083)
    • 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)
    • 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)
    • 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 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)
      • only 5 of 21 homozygotes managed to control their blood loss without cauterization   (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)
  • 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 thrombi   (MGI Ref ID J:49083)

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)
View Research Applications

Research Applications
This mouse can be used to support research in many areas including:

Vwftm1Wgr related

Hematological Research
Clotting Defects

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol Vwftm1Wgr
Allele Name targeted mutation 1, Denisa D Wagner
Allele Type Targeted (Null/Knockout)
Common Name(s) VWF KO; vWf-;
Mutation Made By Cecile Denis,   Center for Blood Research
Strain of Origin129S2/SvPas
ES Cell Line NameD3
ES Cell Line Strain129S2/SvPas
Gene Symbol and Name Vwf, Von Willebrand factor homolog
Chromosome 6
Gene Common Name(s) 6820430P06Rik; AI551257; B130011O06Rik; F8VWF; RIKEN cDNA 6820430P06 gene; RIKEN cDNA B130011O06 gene; VWD; expressed sequence AI551257;
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. [MGI Ref ID J:49083]

Genotyping

Genotyping Information

Genotyping Protocols

Vwftm1Wgr, Separated PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

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: 11154224]  [MGI Ref ID 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: 10930441]  [MGI Ref ID J:63750]

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: 11071623]  [MGI Ref ID 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: 23343833]  [MGI Ref ID 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: 20959603]  [MGI Ref ID 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: 24270421]  [MGI Ref ID 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: 23945153]  [MGI Ref ID 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: 18695007]  [MGI Ref ID 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: 17119108]  [MGI Ref ID 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: 18083848]  [MGI Ref ID J:133469]

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: 21037087]  [MGI Ref ID 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: 20616311]  [MGI Ref ID 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: 18556568]  [MGI Ref ID 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: 9689113]  [MGI Ref ID 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: 11154224]  [MGI Ref ID 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: 15956287]  [MGI Ref ID 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: 17380206]  [MGI Ref ID J:121278]

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: 17996853]  [MGI Ref ID 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: 22983446]  [MGI Ref ID 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: 18256319]  [MGI Ref ID 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: 23812299]  [MGI Ref ID 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: 12526796]  [MGI Ref ID 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: 8918453]  [MGI Ref ID 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: 19182208]  [MGI Ref ID 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: 24553181]  [MGI Ref ID 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: 23335371]  [MGI Ref ID 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: 18487513]  [MGI Ref ID 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: 18187670]  [MGI Ref ID 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: 22855607]  [MGI Ref ID 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: 20194895]  [MGI Ref ID 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: 11520791]  [MGI Ref ID J:106677]

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: 22915646]  [MGI Ref ID 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: 10930441]  [MGI Ref ID 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: 18688020]  [MGI Ref ID J:140334]

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: 17148587]  [MGI Ref ID 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: 18802014]  [MGI Ref ID 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: 20716766]  [MGI Ref ID 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: 23737952]  [MGI Ref ID J:200787]

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: 21346256]  [MGI Ref ID 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: 20200350]  [MGI Ref ID 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: 19036705]  [MGI Ref ID 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: 22267483]  [MGI Ref ID 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: 23797810]  [MGI Ref ID 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: 22134168]  [MGI Ref ID 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: 10688827]  [MGI Ref ID 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: 20360471]  [MGI Ref ID 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: 20606161]  [MGI Ref ID 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: 16823491]  [MGI Ref ID J:111741]

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: 21048155]  [MGI Ref ID 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: 23335368]  [MGI Ref ID J:195866]

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: 19332769]  [MGI Ref ID 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: 16824188]  [MGI Ref ID 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: 15731176]  [MGI Ref ID 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: 19687510]  [MGI Ref ID 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: 18559674]  [MGI Ref ID J:138724]

Health & husbandry

The genotypes of the animals provided may not reflect those discussed in the strain description or the mating scheme utilized by The Jackson Laboratory prior to cryopreservation. Please inquire for possible genotypes for this specific strain.

Health & Colony Maintenance Information

Animal Health Reports

Production of mice from cryopreserved embryos or sperm occurs in a maximum barrier room, G200.

Colony Maintenance

Breeding & HusbandryThis strain originated on a B6;129S2 background and has been backcrossed to C57BL/6J for at least seven generations before it was made homozygous. When held in a live colony, this strain is maintained by homozygous matings.

Pricing and Purchasing

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Cryorecovery* $2525.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $1650.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

Pricing for International shipping destinations View USA Canada and Mexico Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $3283.00
Animals Provided

At least two mice that carry the mutation (if it is a mutant strain) will be provided. Their genotypes may not reflect those discussed in the strain description. Please inquire for possible genotypes and see additional details below.

Frozen Products

Price (US dollars $)
Frozen Embryo $2145.00

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Supply Notes

  • Cryopreserved Embryos
    Available to most shipping destinations1
    This strain is also available as cryopreserved embryos2. Orders for cryopreserved embryos may be placed with our Customer Service Department. Experienced technicians at The Jackson Laboratory have recovered frozen embryos of this strain successfully. We will provide you enough embryos to perform two embryo transfers. The Jackson Laboratory does not guarantee successful recovery at your facility. For complete information on purchasing embryos, please visit our Cryopreserved Embryos web page.

    1 Shipments cannot be made to Australia due to Australian government import restrictions.
    2 Embryos for most strains are cryopreserved at the two cell stage while some strains are cryopreserved at the eight cell stage. If this information is important to you, please contact Customer Service.
  • Cryorecovery - Standard.
    Progeny testing is not required.

    The average number of mice provided from recovery of our cryopreserved strains is 10. The total number of animals provided, their gender and genotype will vary. We will fulfill your order by providing at least two pair of mice, at least one animal of each pair carrying the mutation of interest. Please inquire if larger numbers of animals with specific genotype and genders are needed. Animals typically ship between 10 and 14 weeks from the date of your order. If a second cryorecovery is needed in order to provide the minimum number of animals, animals will ship within 25 weeks. IMPORTANT NOTE: The genotypes of animals provided may not reflect the mating scheme utilized by The Jackson Laboratory prior to cryopreservation, or that discussed in the strain description. Please inquire about possible genotypes which will be recovered for this specific strain. The Jackson Laboratory cannot guarantee the reproductive success of mice shipped to your facility. If the mice are lost after the first three days (post-arrival) or do not produce progeny at your facility, a new order and fee will be necessary.

    Cryorecovery to establish a Dedicated Supply for greater quantities of mice. Mice recovered can be used to establish a dedicated colony to contractually supply you mice according to your requirements. Price by quotation. For more information on Dedicated Supply, please contact JAX® Services, Tel: 1-800-422-6423 (from U.S.A., Canada or Puerto Rico only) or 1-207-288-5845 (from any location).

View USA Canada and Mexico Pricing View International Pricing

Standard Supply

Cryopreserved. Ready for recovery. Please refer to pricing and supply notes on the strain data sheet for further information.

Control Information

  Control
   Wild-type from the colony
   000664 C57BL/6J
 
  Considerations for Choosing Controls
  Control Pricing Information for Genetically Engineered Mutant Strains.
 

Payment Terms and Conditions

Terms are granted by individual review and stated on the customer invoice(s) and account statement. These transactions are payable in U.S. currency within the granted terms. Payment for services, products, shipping containers, and shipping costs that are rendered are expected within the payment terms indicated on the invoice or stated by contract. Invoices and account balances in arrears of stated terms may result in The Jackson Laboratory pursuing collection activities including but not limited to outside agencies and court filings.


See Terms of Use tab for General Terms and Conditions


The Jackson Laboratory's Genotype Promise

The Jackson Laboratory has rigorous genetic quality control and mutant gene genotyping programs to ensure the genetic background of JAX® Mice strains as well as the genotypes of strains with identified molecular mutations. JAX® Mice strains are only made available to researchers after meeting our standards. However, the phenotype of each strain may not be fully characterized and/or captured in the strain data sheets. Therefore, we cannot guarantee a strain's phenotype will meet all expectations. To ensure that JAX® Mice will meet the needs of individual research projects or when requesting a strain that is new to your research, we suggest ordering and performing tests on a small number of mice to determine suitability for your particular project.
Ordering Information
JAX® Mice
Surgical and Preconditioning Services
JAX® Services
Customer Services and Support
Tel: 1-800-422-6423 or 1-207-288-5845
Fax: 1-207-288-6150
Technical Support Email Form

Terms of Use

Terms of Use


General Terms and Conditions


For Licensing and Use Restrictions view the link(s) below:
- Use of MICE by companies or for-profit entities requires a license prior to shipping.

Contact information

General inquiries regarding Terms of Use

Contracts Administration

phone:207-288-6470

JAX® Mice, Products & Services Conditions of Use

"MICE" means mouse strains, their progeny derived by inbreeding or crossbreeding, unmodified derivatives from mouse strains or their progeny supplied by The Jackson Laboratory ("JACKSON"). "PRODUCTS" means biological materials supplied by JACKSON, and their derivatives. "RECIPIENT" means each recipient of MICE, PRODUCTS, or services provided by JACKSON including each institution, its employees and other researchers under its control. MICE or PRODUCTS shall not be: (i) used for any purpose other than the internal research, (ii) sold or otherwise provided to any third party for any use, or (iii) provided to any agent or other third party to provide breeding or other services. Acceptance of MICE or PRODUCTS from JACKSON shall be deemed as agreement by RECIPIENT to these conditions, and departure from these conditions requires JACKSON's prior written authorization.

No Warranty

MICE, PRODUCTS AND SERVICES ARE PROVIDED “AS IS”. JACKSON EXTENDS NO WARRANTIES OF ANY KIND, EITHER EXPRESS, IMPLIED, OR STATUTORY, WITH RESPECT TO MICE, PRODUCTS OR SERVICES, INCLUDING ANY IMPLIED WARRANTY OF MERCHANTABILITY OR FITNESS FOR A PARTICULAR PURPOSE, OR ANY WARRANTY OF NON-INFRINGEMENT OF ANY PATENT, TRADEMARK, OR OTHER INTELLECTUAL PROPERTY RIGHTS.

In case of dissatisfaction for a valid reason and claimed in writing by a purchaser within ninety (90) days of receipt of mice, products or services, JACKSON will, at its option, provide credit or replacement for the mice or product received or the services provided.

No Liability

In no event shall JACKSON, its trustees, directors, officers, employees, and affiliates be liable for any causes of action or damages, including any direct, indirect, special, or consequential damages, arising out of the provision of MICE, PRODUCTS or services, including economic damage or injury to property and lost profits, and including any damage arising from acts or negligence on the part of JACKSON, its agents or employees. Unless prohibited by law, in purchasing or receiving MICE, PRODUCTS or services from JACKSON, purchaser or recipient, or any party claiming by or through them, expressly releases and discharges JACKSON from all such causes of action or damages, and further agrees to defend and indemnify JACKSON from any costs or damages arising out of any third party claims.

MICE and PRODUCTS are to be used in a safe manner and in accordance with all applicable governmental rules and regulations.

The foregoing represents the General Terms and Conditions applicable to JACKSON’s MICE, PRODUCTS or services. In addition, special terms and conditions of sale of certain MICE, PRODUCTS or services may be set forth separately in JACKSON web pages, catalogs, price lists, contracts, and/or other documents, and these special terms and conditions shall also govern the sale of these MICE, PRODUCTS and services by JACKSON, and by its licensees and distributors.

Acceptance of delivery of MICE, PRODUCTS or services shall be deemed agreement to these terms and conditions. No purchase order or other document transmitted by purchaser or recipient that may modify the terms and conditions hereof, shall be in any way binding on JACKSON, and instead the terms and conditions set forth herein, including any special terms and conditions set forth separately, shall govern the sale of MICE, PRODUCTS or services by JACKSON.


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