Strain Name:

B6.129S4-C4btm1Crr/J

Stock Number:

003643

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Availability:

Cryopreserved - Ready for recovery

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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

Former Names B6.129S4-C4tm1Crr/J    (Changed: 21-FEB-06 )
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
Background Strain C57BL/6J
Donor Strain 129S4 via J1 ES cell line
 
Donating Investigator Michael C. Carroll,   The Center for Blood Research

Description
Mice homozygous for the C4 (complement component C4) targeted mutation are viable and fertile. Homozygous mutants exhibit an increased susceptibility to lethal infection by Group B streptococci(GBS). C4 homozygous mutants show a similar susceptibility to GBS lethal infection as do C3 null mice, suggesting that the classical, and not alternative, pathway is primarily involved in antibody-independent humoral immunity to GBS. Homozygotes also demonstrate a profound defect in antibody response to T cell dependent antigens. They show a diminished level of peanut agglutin+ germinal centers and a failure in isotype switching despite normal B cell signalling in vitro.

Control Information

  Control
   See control note: Alternatively, C57BL/6J (Stock No. 000664) mice may be used.
   Wild-type from the colony
 
  Considerations for Choosing Controls

Related Strains

Strains carrying other alleles of C4b
000473   C3H-H2o2 C4bb/SfSnJ
View Strains carrying other alleles of C4b     (1 strain)

Phenotype

Phenotype Information

View Related Disease (OMIM) Terms

Related Disease (OMIM) Terms provided by MGI
- Potential model based on gene homology relationships. Phenotypic similarity to the human disease has not been tested.
Complement Component 4b Deficiency; C4BD   (C4B)
View Mammalian Phenotype Terms

Mammalian Phenotype Terms provided by MGI
      assigned by genotype

The following phenotype information may relate to a genetic background differing from this JAX® Mice strain.

C4btm1Crr/C4btm1Crr

        involves: 129S4/SvJae * C57BL/6
  • mortality/aging
  • increased sensitivity to induced morbidity/mortality
    • caecal ligation and puncture (CLP) causes greater mortality in mutants than wild-type in the first 24 hours after CLP (100 vs 20% mortality)   (MGI Ref ID J:44240)
  • increased susceptibility to bacterial infection induced morbidity/mortality
    • increased mortality from GBS infection compared to controls is limited to the first 3 days after challenge   (MGI Ref ID J:30152)
    • mice challenged with group B Streptococci (GBS) infection become bacteremic within 3 days and die or clear bacteria and survive; C4b-deficient mice challenged with GBS infection showed a decreased LD50 dose compared to immunocompetent controls (LD50 dose: control 6.3 x 104 vs. 2.4 x 103 in C4b-deficient mice)   (MGI Ref ID J:30152)
  • immune system phenotype
  • *normal* immune system phenotype
    • serum from C4-deficient mice has similar antibody-mediated opsonophagocytic killing of GBS to control serum   (MGI Ref ID J:30152)
    • secondary immune responses are similar in wild type and mutant mice, so helper T cell function is normal secondary immune responses are similar in wild-type and mutant mice, so helper T cell function is normal   (MGI Ref ID J:64282)
    • B cells from deficient mice show normal proliferative effects in response to surface IgM crosslinking   (MGI Ref ID J:64282)
    • abnormal humoral immune response
      • when immunized with a 10-fold higher amount of bacteriophage, mice show a weak IgG response but response is still 10-fold lower than wild-type   (MGI Ref ID J:64282)
      • in response to immunization with bacteriophage (phiX174), a T cell dependent antigen, C4b-deficient mice mount a weak Ig M response but fail to switch to IgG   (MGI Ref ID J:64282)
    • abnormal spleen germinal center morphology   (MGI Ref ID J:64282)
      • decreased spleen germinal center number
        • after immunization, wild-type mice develop germinal centers (GC) in ~half of splenic follicles while ~10% of splenic follicles in C3-deficient mice contain GCs   (MGI Ref ID J:64282)
      • decreased spleen germinal center size
        • diameter of GCs are less than that observed in wild-type   (MGI Ref ID J:64282)
    • decreased susceptibility to bacterial infection
      • when pregnant dams are immunized with immune rabbit serum, pups are protected against lethal challenge on day 2 of life because of transfer of maternal antibodies to the pups (15/16 pups survive, similar to controls)   (MGI Ref ID J:30152)
    • increased susceptibility to bacterial infection induced morbidity/mortality
      • increased mortality from GBS infection compared to controls is limited to the first 3 days after challenge   (MGI Ref ID J:30152)
      • mice challenged with group B Streptococci (GBS) infection become bacteremic within 3 days and die or clear bacteria and survive; C4b-deficient mice challenged with GBS infection showed a decreased LD50 dose compared to immunocompetent controls (LD50 dose: control 6.3 x 104 vs. 2.4 x 103 in C4b-deficient mice)   (MGI Ref ID J:30152)
    • liver inflammation
      • mild hepatitis is observed in a small number (1/11) of mice surviving systemic challenge with GBS doses of 102 to 105 CFU upon necropsy 15 days post-challenge; there are aggregates of mononuclear or polymorphonuclear leukocytes in hepatic sinusoids; wild-type animals that receive higher doses of GBS showed similar lesions   (MGI Ref ID J:30152)
  • homeostasis/metabolism phenotype
  • *normal* homeostasis/metabolism phenotype
    • response to renal ischemia reperfusion injury is the same as in wild-type mice   (MGI Ref ID J:120567)
  • hematopoietic system phenotype
  • abnormal spleen germinal center morphology   (MGI Ref ID J:64282)
    • decreased spleen germinal center number
      • after immunization, wild-type mice develop germinal centers (GC) in ~half of splenic follicles while ~10% of splenic follicles in C3-deficient mice contain GCs   (MGI Ref ID J:64282)
    • decreased spleen germinal center size
      • diameter of GCs are less than that observed in wild-type   (MGI Ref ID J:64282)
  • cardiovascular system phenotype
  • decreased vascular permeability
    • upeon reperfusion of ischemic intestine (jejunum), permeability index (PI) of injured C4b-deficient mice is reduced compared to control treated wild-type (PI of 2.20 vs 3.26 in controls)   (MGI Ref ID J:78613)
  • digestive/alimentary phenotype
  • abnormal intestine morphology
    • mice show less evidence of infarction compared to controls   (MGI Ref ID J:78613)
  • liver/biliary system phenotype
  • liver inflammation
    • mild hepatitis is observed in a small number (1/11) of mice surviving systemic challenge with GBS doses of 102 to 105 CFU upon necropsy 15 days post-challenge; there are aggregates of mononuclear or polymorphonuclear leukocytes in hepatic sinusoids; wild-type animals that receive higher doses of GBS showed similar lesions   (MGI Ref ID J:30152)

C4btm1Crr/C4btm1Crr

        involves: 129S4/SvJae
  • renal/urinary system phenotype
  • glomerulonephritis
    • reduced neutrophil infiltration of glomerulus relative to controls when injected with 0.5 mg and with 1.0mg of anti-glomerular basement membrane antiserum   (MGI Ref ID J:44715)
  • homeostasis/metabolism phenotype
  • abnormal blood coagulation
    • thrombus formation only moderately reduced   (MGI Ref ID J:44715)
  • immune system phenotype
  • glomerulonephritis
    • reduced neutrophil infiltration of glomerulus relative to controls when injected with 0.5 mg and with 1.0mg of anti-glomerular basement membrane antiserum   (MGI Ref ID J:44715)
View Research Applications

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

C4btm1Crr related

Developmental Biology Research
Lymphoid Tissue Defects
      hematopoietic defects

Immunology, Inflammation and Autoimmunity Research
Immunodeficiency
      specific complement deficiency
Immunodeficiency Associated with Other Defects
Inflammation

Research Tools
Immunology, Inflammation and Autoimmunity Research
      specific complement deficiency

Genes & Alleles

Gene & Allele Information provided by MGI

 
Allele Symbol C4btm1Crr
Allele Name targeted mutation 1, Michael C Carroll
Allele Type Targeted (knock-out)
Common Name(s) C4-;
Mutation Made By Michael Carroll,   The Center for Blood Research
Strain of Origin129S4/SvJae
ES Cell Line NameJ1
ES Cell Line Strain129S4/SvJae
Gene Symbol and Name C4b, complement component 4B (Chido blood group)
Chromosome 17
Gene Common Name(s) C4; C4-1; C4-2; C4A2; C4A3; C4A4; C4A6; C4AD; C4B1; C4B12; C4B2; C4B3; C4B5; C4BD; C4B_2; C4F; C4S; C4l; CH; CO4; CPAMD2; CPAMD3; RG; Ss; complement component 4 (within H-2S); serum substance;
Molecular Note A genomic fragment corresponding to 987 nucleotides of coding sequence (exons 23-29) was replaced with a neomycin selection cassette. ELISA assays on serum derived from homozygous mice demonstrated that no detectable protein is produced from this allele. [MGI Ref ID J:64282]

Genotyping

Genotyping Information

Genotyping Protocols

C4btm1Crralternate1, Standard PCR
C4btm1Crr, Standard PCR


Helpful Links

Genotyping resources and troubleshooting

References

References provided by MGI

Selected Reference(s)

Fischer MB; Ma M; Goerg S; Zhou X; Xia J; Finco O; Han S; Kelsoe G; Howard RG; Rothstein TL; Kremmer E; Rosen FS; Carroll MC. 1996. Regulation of the B cell response to T-dependent antigens by classical pathway complement. J Immunol 157(2):549-56. [PubMed: 8752901]  [MGI Ref ID J:64282]

Additional References

Tuzun E; Scott BG; Goluszko E; Higgs S; Christadoss P. 2003. Genetic evidence for involvement of classical complement pathway in induction of experimental autoimmune myasthenia gravis. J Immunol 171(7):3847-54. [PubMed: 14500686]  [MGI Ref ID J:85645]

C4btm1Crr related

Abonia JP; Friend DS; Austen WG Jr; Moore FD Jr; Carroll MC; Chan R; Afnan J; Humbles A; Gerard C; Knight P; Kanaoka Y; Yasuda S; Morokawa N; Austen KF; Stevens RL; Gurish MF. 2005. Mast cell protease 5 mediates ischemia-reperfusion injury of mouse skeletal muscle. J Immunol 174(11):7285-91. [PubMed: 15905575]  [MGI Ref ID J:98963]

Ali YM; Lynch NJ; Haleem KS; Fujita T; Endo Y; Hansen S; Holmskov U; Takahashi K; Stahl GL; Dudler T; Girija UV; Wallis R; Kadioglu A; Stover CM; Andrew PW; Schwaeble WJ. 2012. The lectin pathway of complement activation is a critical component of the innate immune response to pneumococcal infection. PLoS Pathog 8(7):e1002793. [PubMed: 22792067]  [MGI Ref ID J:195379]

Banda NK; Takahashi M; Levitt B; Glogowska M; Nicholas J; Takahashi K; Stahl GL; Fujita T; Arend WP; Holers VM. 2010. Essential role of complement mannose-binding lectin-associated serine proteases-1/3 in the murine collagen antibody-induced model of inflammatory arthritis. J Immunol 185(9):5598-606. [PubMed: 20870940]  [MGI Ref ID J:165200]

Banda NK; Takahashi M; Takahashi K; Stahl GL; Hyatt S; Glogowska M; Wiles TA; Endo Y; Fujita T; Michael Holers V; Arend WP. 2011. Mechanisms of mannose-binding lectin-associated serine proteases-1/3 activation of the alternative pathway of complement. Mol Immunol 49(1-2):281-9. [PubMed: 21943708]  [MGI Ref ID J:177350]

Banda NK; Thurman JM; Kraus D; Wood A; Carroll MC; Arend WP; Holers VM. 2006. Alternative complement pathway activation is essential for inflammation and joint destruction in the passive transfer model of collagen-induced arthritis. J Immunol 177(3):1904-12. [PubMed: 16849503]  [MGI Ref ID J:138361]

Baudino L; Sardini A; Ruseva MM; Fossati-Jimack L; Cook HT; Scott D; Simpson E; Botto M. 2014. C3 opsonization regulates endocytic handling of apoptotic cells resulting in enhanced T-cell responses to cargo-derived antigens. Proc Natl Acad Sci U S A 111(4):1503-8. [PubMed: 24474777]  [MGI Ref ID J:206648]

Bergthaler A; Flatz L; Verschoor A; Hegazy AN; Holdener M; Fink K; Eschli B; Merkler D; Sommerstein R; Horvath E; Fernandez M; Fitsche A; Senn BM; Verbeek JS; Odermatt B; Siegrist CA; Pinschewer DD. 2009. Impaired antibody response causes persistence of prototypic T cell-contained virus. PLoS Biol 7(4):e1000080. [PubMed: 19355789]  [MGI Ref ID J:150498]

Boos LA; Szalai AJ; Barnum SR. 2005. Murine complement C4 is not required for experimental autoimmune encephalomyelitis. Glia 49(1):158-60. [PubMed: 15390104]  [MGI Ref ID J:104909]

Bora NS; Kaliappan S; Jha P; Xu Q; Sohn JH; Dhaulakhandi DB; Kaplan HJ; Bora PS. 2006. Complement activation via alternative pathway is critical in the development of laser-induced choroidal neovascularization: role of factor B and factor H. J Immunol 177(3):1872-8. [PubMed: 16849499]  [MGI Ref ID J:138026]

Chatterjee P; Agyemang AF; Alimzhanov MB; Degn S; Tsiftsoglou SA; Alicot E; Jones SA; Ma M; Carroll MC. 2013. Complement C4 maintains peripheral B-cell tolerance in a myeloid cell dependent manner. Eur J Immunol 43(9):2441-50. [PubMed: 23749435]  [MGI Ref ID J:201329]

Chen Z; Koralov SB; Kelsoe G. 2000. Complement C4 inhibits systemic autoimmunity through a mechanism independent of complement receptors CR1 and CR2. J Exp Med 192(9):1339-52. [PubMed: 11067882]  [MGI Ref ID J:111811]

Chiu IM; Phatnani H; Kuligowski M; Tapia JC; Carrasco MA; Zhang M; Maniatis T; Carroll MC. 2009. Activation of innate and humoral immunity in the peripheral nervous system of ALS transgenic mice. Proc Natl Acad Sci U S A 106(49):20960-5. [PubMed: 19933335]  [MGI Ref ID J:155542]

Clark A; Weymann A; Hartman E; Turmelle Y; Carroll M; Thurman JM; Holers VM; Hourcade DE; Rudnick DA. 2008. Evidence for non-traditional activation of complement factor C3 during murine liver regeneration. Mol Immunol 45(11):3125-32. [PubMed: 18452991]  [MGI Ref ID J:136225]

Cunnion KM; Benjamin DK Jr; Hester CG; Frank MM. 2004. Role of complement receptors 1 and 2 (CD35 and CD21), C3, C4, and C5 in survival by mice of Staphylococcus aureus bacteremia. J Lab Clin Med 143(6):358-65. [PubMed: 15192652]  [MGI Ref ID J:101948]

Einav S; Pozdnyakova OO; Ma M; Carroll MC. 2002. Complement C4 is protective for lupus disease independent of C3. J Immunol 168(3):1036-41. [PubMed: 11801636]  [MGI Ref ID J:127292]

Faust KB; Finke D; Klempt-Giessing K; Randers K; Zachrau B; Schlenke P; Kirchner H; Goerg S. 2007. Antigen-induced B cell apoptosis is independent of complement C4. Clin Exp Immunol 150(1):132-9. [PubMed: 17645767]  [MGI Ref ID J:125271]

Finke D; Randers K; Hoerster R; Hennig H; Zawatzky R; Marion T; Brockmann C; Klempt-Giessing K; Jacobsen K; Kirchner H; Goerg S. 2007. Elevated levels of endogenous apoptotic DNA and IFN-alpha in complement C4-deficient mice: implications for induction of systemic lupus erythematosus. Eur J Immunol 37(6):1702-9. [PubMed: 17506029]  [MGI Ref ID J:123513]

Fischer MB; Prodeus AP; Nicholson-Weller A; Ma M; Murrow J; Reid RR; Warren HB; Lage AL; Moore FD Jr; Rosen FS; Carroll MC. 1997. Increased susceptibility to endotoxin shock in complement C3- and C4-deficient mice is corrected by C1 inhibitor replacement. J Immunol 159(2):976-82. [PubMed: 9218619]  [MGI Ref ID J:78654]

Gaspal FM; McConnell FM; Kim MY; Gray D; Kosco-Vilbois MH; Raykundalia CR; Botto M; Lane PJ. 2006. The generation of thymus-independent germinal centers depends on CD40 but not on CD154, the T cell-derived CD40-ligand. Eur J Immunol 36(7):1665-73. [PubMed: 16783845]  [MGI Ref ID J:115798]

Girardi G; Berman J; Redecha P; Spruce L; Thurman JM; Kraus D; Hollmann TJ; Casali P; Caroll MC; Wetsel RA; Lambris JD; Holers VM; Salmon JE. 2003. Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome. J Clin Invest 112(11):1644-54. [PubMed: 14660741]  [MGI Ref ID J:86845]

Gupta VA; Hermiston ML; Cassafer G; Daikh DI; Weiss A. 2008. B cells drive lymphocyte activation and expansion in mice with the CD45 wedge mutation and Fas deficiency. J Exp Med 205(12):2755-2761. [PubMed: 19001138]  [MGI Ref ID J:141398]

Haas KM; Poe JC; Tedder TF. 2009. CD21/35 promotes protective immunity to Streptococcus pneumoniae through a complement-independent but CD19-dependent pathway that regulates PD-1 expression. J Immunol 183(6):3661-71. [PubMed: 19710450]  [MGI Ref ID J:152400]

Hebert MJ; Takano T; Papayianni A; Rennke HG; Minto A; Salant DJ; Carroll MC; Brady HR. 1998. Acute nephrotoxic serum nephritis in complement knockout mice: relative roles of the classical and alternate pathways in neutrophil recruitment and proteinuria. Nephrol Dial Transplant 13(11):2799-803. [PubMed: 9829481]  [MGI Ref ID J:77011]

Heimbach L; Li Z; Berkowitz P; Zhao M; Li N; Rubenstein DS; Diaz LA; Liu Z. 2011. The C5a Receptor on Mast Cells Is Critical for the Autoimmune Skin-blistering Disease Bullous Pemphigoid. J Biol Chem 286(17):15003-9. [PubMed: 21393236]  [MGI Ref ID J:172081]

Kang YS; Do Y; Lee HK; Park SH; Cheong C; Lynch RM; Loeffler JM; Steinman RM; Park CG. 2006. A dominant complement fixation pathway for pneumococcal polysaccharides initiated by SIGN-R1 interacting with C1q. Cell 125(1):47-58. [PubMed: 16615889]  [MGI Ref ID J:115872]

Kim DD; Miwa T; Kimura Y; Schwendener RA; van Lookeren Campagne M; Song WC. 2008. Deficiency of decay-accelerating factor and complement receptor 1-related gene/protein y on murine platelets leads to complement-dependent clearance by the macrophage phagocytic receptor CRIg. Blood 112(4):1109-19. [PubMed: 18524992]  [MGI Ref ID J:138410]

Lin T; Zhou W; Farrar CA; Hargreaves RE; Sheerin NS; Sacks SH. 2006. Deficiency of c4 from donor or recipient mouse fails to prevent renal allograft rejection. Am J Pathol 168(4):1241-8. [PubMed: 16565498]  [MGI Ref ID J:108204]

Manderson AP; Pickering MC; Botto M; Walport MJ; Parish CR. 2001. Continual low-level activation of the classical complement pathway. J Exp Med 194(6):747-56. [PubMed: 11560991]  [MGI Ref ID J:119459]

Mao C; Jiang L; Melo-Jorge M; Puthenveetil M; Zhang X; Carroll MC; Imanishi-Kari T. 2004. T cell-independent somatic hypermutation in murine B cells with an immature phenotype. Immunity 20(2):133-44. [PubMed: 14975236]  [MGI Ref ID J:89757]

Mao D; Wu X; Deppong C; Friend LD; Dolecki G; Nelson DM; Molina H. 2003. Negligible role of antibodies and C5 in pregnancy loss associated exclusively with C3-dependent mechanisms through complement alternative pathway. Immunity 19(6):813-22. [PubMed: 14670299]  [MGI Ref ID J:86999]

Markiewski MM; DeAngelis RA; Benencia F; Ricklin-Lichtsteiner SK; Koutoulaki A; Gerard C; Coukos G; Lambris JD. 2008. Modulation of the antitumor immune response by complement. Nat Immunol 9(11):1225-35. [PubMed: 18820683]  [MGI Ref ID J:143335]

Mattsson J; Yrlid U; Stensson A; Schon K; Karlsson MC; Ravetch JV; Lycke NY. 2011. Complement activation and complement receptors on follicular dendritic cells are critical for the function of a targeted adjuvant. J Immunol 187(7):3641-52. [PubMed: 21880985]  [MGI Ref ID J:179328]

Mehlhop E; Diamond MS. 2006. Protective immune responses against West Nile virus are primed by distinct complement activation pathways. J Exp Med 203(5):1371-81. [PubMed: 16651386]  [MGI Ref ID J:124138]

Miwa T; Sato S; Gullipalli D; Nangaku M; Song WC. 2013. Blocking Properdin, the Alternative Pathway, and Anaphylatoxin Receptors Ameliorates Renal Ischemia-Reperfusion Injury in Decay-Accelerating Factor and CD59 Double-Knockout Mice. J Immunol 190(7):3552-9. [PubMed: 23427256]  [MGI Ref ID J:194520]

Mold C; Rodic-Polic B; Du Clos TW. 2002. Protection from Streptococcus pneumoniae infection by C-reactive protein and natural antibody requires complement but not Fc gamma receptors. J Immunol 168(12):6375-81. [PubMed: 12055255]  [MGI Ref ID J:123793]

Moller-Kristensen M; Hamblin MR; Thiel S; Jensenius JC; Takahashi K. 2007. Burn injury reveals altered phenotype in mannan-binding lectin-deficient mice. J Invest Dermatol 127(6):1524-31. [PubMed: 17363917]  [MGI Ref ID J:121549]

Moulton EA; Atkinson JP; Buller RM. 2008. Surviving mousepox infection requires the complement system. PLoS Pathog 4(12):e1000249. [PubMed: 19112490]  [MGI Ref ID J:162201]

Mueller-Ortiz SL; Drouin SM; Wetsel RA. 2004. The alternative activation pathway and complement component C3 are critical for a protective immune response against Pseudomonas aeruginosa in a murine model of pneumonia. Infect Immun 72(5):2899-906. [PubMed: 15102802]  [MGI Ref ID J:89480]

Nelson KC; Zhao M; Schroeder PR; Li N; Wetsel RA; Diaz LA; Liu Z. 2006. Role of different pathways of the complement cascade in experimental bullous pemphigoid. J Clin Invest 116(11):2892-2900. [PubMed: 17024247]  [MGI Ref ID J:114506]

Nimmerjahn F; Ravetch JV. 2005. Divergent immunoglobulin g subclass activity through selective Fc receptor binding. Science 310(5753):1510-2. [PubMed: 16322460]  [MGI Ref ID J:103229]

Ochsenbein AF; Pinschewer DD; Odermatt B; Carroll MC; Hengartner H; Zinkernagel RM. 1999. Protective T cell-independent antiviral antibody responses are dependent on complement. J Exp Med 190(8):1165-74. [PubMed: 10523614]  [MGI Ref ID J:115126]

Otten MA; Groeneveld TW; Flierman R; Rastaldi MP; Trouw LA; Faber-Krol MC; Visser A; Essers MC; Claassens J; Verbeek JS; van Kooten C; Roos A; Daha MR. 2009. Both complement and IgG fc receptors are required for development of attenuated antiglomerular basement membrane nephritis in mice. J Immunol 183(6):3980-8. [PubMed: 19710463]  [MGI Ref ID J:152300]

Paul E; Pozdnyakova OO; Mitchell E; Carroll MC. 2002. Anti-DNA autoreactivity in C4-deficient mice. Eur J Immunol 32(9):2672-9. [PubMed: 12207352]  [MGI Ref ID J:78993]

Peng Q; Li K; Anderson K; Farrar CA; Lu B; Smith RA; Sacks SH; Zhou W. 2008. Local production and activation of complement up-regulates the allostimulatory function of dendritic cells through C3a-C3aR interaction. Blood 111(4):2452-61. [PubMed: 18056835]  [MGI Ref ID J:131316]

Prodeus AP; Zhou X; Maurer M; Galli SJ; Carroll MC. 1997. Impaired mast cell-dependent natural immunity in complement C3-deficient mice. Nature 390(6656):172-5. [PubMed: 9367154]  [MGI Ref ID J:44240]

Quartier P; Potter PK; Ehrenstein MR; Walport MJ; Botto M. 2005. Predominant role of IgM-dependent activation of the classical pathway in the clearance of dying cells by murine bone marrow-derived macrophages in vitro. Eur J Immunol 35(1):252-60. [PubMed: 15597324]  [MGI Ref ID J:95231]

Quigg RJ; Lim A; Haas M; Alexander JJ; He C; Carroll MC. 1998. Immune complex glomerulonephritis in C4- and C3-deficient mice. Kidney Int 53(2):320-30. [PubMed: 9461092]  [MGI Ref ID J:47055]

Renner B; Strassheim D; Amura CR; Kulik L; Ljubanovic D; Glogowska MJ; Takahashi K; Carroll MC; Holers VM; Thurman JM. 2010. B cell subsets contribute to renal injury and renal protection after ischemia/reperfusion. J Immunol 185(7):4393-400. [PubMed: 20810984]  [MGI Ref ID J:164313]

Schwaeble WJ; Lynch NJ; Clark JE; Marber M; Samani NJ; Ali YM; Dudler T; Parent B; Lhotta K; Wallis R; Farrar CA; Sacks S; Lee H; Zhang M; Iwaki D; Takahashi M; Fujita T; Tedford CE; Stover CM. 2011. Targeting of mannan-binding lectin-associated serine protease-2 confers protection from myocardial and gastrointestinal ischemia/reperfusion injury. Proc Natl Acad Sci U S A 108(18):7523-8. [PubMed: 21502512]  [MGI Ref ID J:172218]

Sheerin NS; Springall T; Carroll MC; Hartley B; Sacks SH. 1997. Protection against anti-glomerular basement membrane (GBM)-mediated nephritis in C3- and C4-deficient mice. Clin Exp Immunol 110(3):403-9. [PubMed: 9409643]  [MGI Ref ID J:44715]

Solomon S; Kolb C; Mohanty S; Jeisy-Walder E; Preyer R; Schollhorn V; Illges H. 2002. Transmission of antibody-induced arthritis is independent of complement component 4 (C4) and the complement receptors 1 and 2 (CD21/35). Eur J Immunol 32(3):644-51. [PubMed: 11857338]  [MGI Ref ID J:115361]

Sponarova J; Nuvolone M; Whicher C; Frei N; Kana V; Schwarz P; Westermark GT; Aguzzi A. 2013. Efficient amyloid a clearance in the absence of immunoglobulins and complement factors. Am J Pathol 182(4):1297-307. [PubMed: 23454183]  [MGI Ref ID J:195307]

Springall T; Sheerin NS; Abe K; Holers VM; Wan H; Sacks SH. 2001. Epithelial secretion of C3 promotes colonization of the upper urinary tract by Escherichia coli. Nat Med 7(7):801-6. [PubMed: 11433344]  [MGI Ref ID J:70251]

Suber F; Carroll MC; Moore FD Jr. 2007. Innate response to self-antigen significantly exacerbates burn wound depth. Proc Natl Acad Sci U S A 104(10):3973-7. [PubMed: 17360462]  [MGI Ref ID J:120063]

Takahashi M; Ishida Y; Iwaki D; Kanno K; Suzuki T; Endo Y; Homma Y; Fujita T. 2010. Essential role of mannose-binding lectin-associated serine protease-1 in activation of the complement factor D. J Exp Med 207(1):29-37, S1-3. [PubMed: 20038603]  [MGI Ref ID J:156544]

Tang T; Rosenkranz A; Assmann KJM; Goodman MJ; Gutierrez-Ramos JC ; Carroll MC ; Cotran RS ; Mayadas TN. 1997. A role for Mac-1 (CDIIb/CD18) in immune complex-stimulated neutrophil function in vivo: Mac-1 deficiency abrogates sustained Fcgamma receptor-dependent neutrophil adhesion and complement-dependent proteinuria in acute glomerulonephritis. J Exp Med 186(11):1853-63. [PubMed: 9382884]  [MGI Ref ID J:44403]

Taube C; Thurman JM; Takeda K; Joetham A; Miyahara N; Carroll MC; Dakhama A; Giclas PC; Holers VM; Gelfand EW. 2006. Factor B of the alternative complement pathway regulates development of airway hyperresponsiveness and inflammation. Proc Natl Acad Sci U S A 103(21):8084-9. [PubMed: 16702544]  [MGI Ref ID J:110220]

Taylor PR; Carugati A; Fadok VA; Cook HT; Andrews M; Carroll MC; Savill JS; Henson PM; Botto M; Walport MJ. 2000. A hierarchical role for classical pathway complement proteins in the clearance of apoptotic cells in vivo. J Exp Med 192(3):359-66. [PubMed: 10934224]  [MGI Ref ID J:63875]

Trendelenburg M; Fossati-Jimack L; Cortes-Hernandez J; Turnberg D; Lewis M; Izui S; Cook HT; Botto M. 2005. The role of complement in cryoglobulin-induced immune complex glomerulonephritis. J Immunol 175(10):6909-14. [PubMed: 16272350]  [MGI Ref ID J:119691]

Tuzun E; Scott BG; Goluszko E; Higgs S; Christadoss P. 2003. Genetic evidence for involvement of classical complement pathway in induction of experimental autoimmune myasthenia gravis. J Immunol 171(7):3847-54. [PubMed: 14500686]  [MGI Ref ID J:85645]

Verschoor A; Neuenhahn M; Navarini AA; Graef P; Plaumann A; Seidlmeier A; Nieswandt B; Massberg S; Zinkernagel RM; Hengartner H; Busch DH. 2011. A platelet-mediated system for shuttling blood-borne bacteria to CD8alpha+ dendritic cells depends on glycoprotein GPIb and complement C3. Nat Immunol 12(12):1194-201. [PubMed: 22037602]  [MGI Ref ID J:179014]

Welch TR; Frenzke M; Carroll MC; Witte DP. 2001. Evidence of a role for C4 in modulating interstitial inflammation in experimental glomerulonephritis. Clin Immunol 101(3):366-70. [PubMed: 11726230]  [MGI Ref ID J:115418]

Wessels MR; Butko P; Ma M; Warren HB; Lage AL; Carroll MC. 1995. Studies of group B streptococcal infection in mice deficient in complement component C3 or C4 demonstrate an essential role for complement in both innate and acquired immunity. Proc Natl Acad Sci U S A 92(25):11490-4. [PubMed: 8524789]  [MGI Ref ID J:30152]

Williams JP; Pechet TT; Weiser MR; Reid R; Kobzik L; Moore FD Jr; Carroll MC; Hechtman HB. 1999. Intestinal reperfusion injury is mediated by IgM and complement. J Appl Physiol 86(3):938-42. [PubMed: 10066708]  [MGI Ref ID J:78613]

Xiao H; Schreiber A; Heeringa P; Falk RJ; Jennette JC. 2007. Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies. Am J Pathol 170(1):52-64. [PubMed: 17200182]  [MGI Ref ID J:117048]

Zabel MD; Heikenwalder M; Prinz M; Arrighi I; Schwarz P; Kranich J; von Teichman A; Haas KM; Zeller N; Tedder TF; Weis JH; Aguzzi A. 2007. Stromal complement receptor CD21/35 facilitates lymphoid prion colonization and pathogenesis. J Immunol 179(9):6144-52. [PubMed: 17947689]  [MGI Ref ID J:152995]

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Health & husbandry

Health & Colony Maintenance Information

Animal Health Reports

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

Pricing and Purchasing

Pricing, Supply Level & Notes, Controls


Pricing for USA, Canada and Mexico shipping destinations View International Pricing

Cryopreserved

Cryopreserved Mice - Ready for Recovery

Price (US dollars $)
Cryorecovery* $2450.00
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.

Embryos

Price (US dollars $)
Frozen Embryo $1600.00

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 11 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* $3185.00
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.

Embryos

Price (US dollars $)
Frozen Embryo $2080.00

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 11 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
   See control note: Alternatively, C57BL/6J (Stock No. 000664) mice may be used.
   Wild-type from the colony
 
  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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