Gene Symbol: C2
Description: complement component 2 (within H-2S)
Alias: complement C2, MHC complement component C2, classical-complement pathway C3/C5 convertase, complement component C2, complement factor C2
Species: mouse
Products:     C2

Top Publications

  1. Yang Z, Yu C. Organizations and gene duplications of the human and mouse MHC complement gene clusters. Exp Clin Immunogenet. 2000;17:1-17 pubmed
    ..The modular duplications of human and mouse RP-C4-CYP21-TNX (RCCX) are sharply different as SKI2W2 is absent in the human MCGC, and TNXA and RP2 are smaller in size but higher in sequence conservation in humans. ..
  2. Peelman L, Mattheeuws M, Van Zeveren A, Van de Weghe A, Bouquet Y. Conservation of the RD-BF-C2 organization in the pig MHC class-III region: mapping and cloning of the pig RD gene. Anim Genet. 1996;27:35-42 pubmed
    ..pulsed field gel electrophoresis identified common 220 kb NruI, 130 kb EagI and 200 kb MluI bands for RD, BF and C2. The RD gene has also a 17 kb Kp nI and 11 kb SacI fragment in common with BF but not with C2...
  3. Ramos T, Darley M, Weckbach S, Stahel P, Tomlinson S, Barnum S. The C5 convertase is not required for activation of the terminal complement pathway in murine experimental cerebral malaria. J Biol Chem. 2012;287:24734-8 pubmed publisher
    ..Our data indicate that activation of C5 in ECM likely occurs via coagulation enzymes of the extrinsic protease pathway. ..
  4. Li Q, Li Y, Stahl G, Thurman J, He Y, Tong H. Essential role of factor B of the alternative complement pathway in complement activation and opsonophagocytosis during acute pneumococcal otitis media in mice. Infect Immun. 2011;79:2578-85 pubmed publisher
    ..Using mice deficient in complement C1qa, factor B, and factor B/C2, we found that complement C3 activation and opsonophagocytosis of S...
  5. Thoren L, Nørgaard G, Weischenfeldt J, Waage J, Jakobsen J, Damgaard I, et al. UPF2 is a critical regulator of liver development, function and regeneration. PLoS ONE. 2010;5:e11650 pubmed publisher
    ..Collectively, our data demonstrate the critical role of the NMD pathway in liver development, function and regeneration and highlights the importance of NMD for mammalian biology. ..
  6. Robson M, Cook H, Botto M, Taylor P, Busso N, Salvi R, et al. Accelerated nephrotoxic nephritis is exacerbated in C1q-deficient mice. J Immunol. 2001;166:6820-8 pubmed
    ..This exacerbated thrombosis was also seen in mice triply deficient in C1q, factor B, and C2, excluding a major pathogenic role for the alternative pathway of complement in this phenomenon...
  7. Mitchell D, Taylor P, Cook H, Moss J, Bygrave A, Walport M, et al. Cutting edge: C1q protects against the development of glomerulonephritis independently of C3 activation. J Immunol. 1999;162:5676-9 pubmed
    ..the contribution of C3 activation to the induction of spontaneous GN, C1qa-/- mice were crossed with factor B- and C2-deficient (H2-Bf/C2-/-) mice...
  8. Taylor P, Nash J, Theodoridis E, Bygrave A, Walport M, Botto M. A targeted disruption of the murine complement factor B gene resulting in loss of expression of three genes in close proximity, factor B, C2, and D17H6S45. J Biol Chem. 1998;273:1699-704 pubmed
    ..Further studies revealed that the two adjacent genes, complement component C2 and D17H6S45, had been down regulated as a result of the disruption...
  9. Kuroda N, Wada H, Naruse K, Simada A, Shima A, Sasaki M, et al. Molecular cloning and linkage analysis of the Japanese medaka fish complement Bf/C2 gene. Immunogenetics. 1996;44:459-67 pubmed
    Evolutionary studies of complement factor B (Bf) and C2 in lower vertebrates have revealed the presence of the Bf/C2 common ancestor-like molecule in lamprey (cyclostome) and the Bf molecule encoded by the duplicated genes closely linked ..

More Information


  1. Takayama Y, Takada F, Takahashi A, Kawakami M. A 100-kDa protein in the C4-activating component of Ra-reactive factor is a new serine protease having module organization similar to C1r and C1s. J Immunol. 1994;152:2308-16 pubmed
    ..RaRF), a C-dependent bactericidal factor in mice, is composed of one polysaccharide-binding component and one C4/C2-activating component...
  2. Lafuse W, Castle L, David C. Restriction fragment length polymorphism analysis of factor B and C2 genes in H-2 recombinant mouse strains. Immunogenetics. 1990;31:275-7 pubmed
  3. Takahashi S, Fukuoka Y, Moriwaki K, Okuda T, Tachibana T, Natsuume Sakai S, et al. Structural polymorphism of mouse complement C2 detected by microscale peptide mapping: linkage to H-2. Immunogenetics. 1984;19:493-501 pubmed
    b>Complement C2 was isolated from 17 mouse strains by immunoprecipitation and sodium dodecyl sulfate-polyacrylamide gel electrophoresis and examined for structural polymorphism by using micro-peptide mapping...
  4. Falus A, Wakeland E, McConnell T, Gitlin J, Whitehead A, Colten H. DNA polymorphism of MHC III genes in inbred and wild mouse strains. Immunogenetics. 1987;25:290-8 pubmed
    Genes encoding the second component (C2), factor B, and complement protein C4 and Slp (sex-limited protein) are members of the major histocompatibility complex class III gene cluster...
  5. Gorman J, Jackson R, Desantola J, Shreffler D, Atkinson J. Development of a hemolytic assay for mouse C2 and determination of its genetic control. J Immunol. 1980;125:344-51 pubmed
  6. Tosi M, Levi Strauss M, Georgatsou E, Amor M, Meo T. Duplications of complement and non-complement genes of the H-2S region: evolutionary aspects of the C4 isotypes and molecular analysis of their expression variants. Immunol Rev. 1985;87:151-83 pubmed
  7. Muller U, Stephan D, Philippsen P, Steinmetz M. Orientation and molecular map position of the complement genes in the mouse MHC. EMBO J. 1987;6:369-73 pubmed
    ..maps orients the complement gene clusters in the S region with the 21-OHB gene pointing towards the K end and the C2 gene pointing towards the D end of the MHC...
  8. Ishikawa N, Nonaka M, Wetsel R, Colten H. Murine complement C2 and factor B genomic and cDNA cloning reveals different mechanisms for multiple transcripts of C2 and B. J Biol Chem. 1990;265:19040-6 pubmed
    ..cDNA clones were isolated to ascertain the mechanisms accounting for previously recognized multiple forms of complement C2 and factor B mRNA and to analyze structural similarities with the corresponding human gene products (C2, 74% ..
  9. Cortese Hassett A, Radojcic A, Watters J, Locker J, Kunz H, Gill T. Mapping and sequencing analysis of the rat MHC. Transplant Proc. 1989;21:3244-6 pubmed
  10. Shin D, Webb B, Nakao M, Smith S. Molecular cloning, structural analysis and expression of complement component Bf/C2 genes in the nurse shark, Ginglymostoma cirratum. Dev Comp Immunol. 2007;31:1168-82 pubmed
    Factor B and C2 are serine proteases that provide the catalytic subunits of C3 and C5 convertases of the alternative (AP) and classical (CP) complement pathways...
  11. Kumanovics A, Lindahl K. G7c in the lung tumor susceptibility (Lts) region of the Mhc class III region encodes a von Willebrand factor type A domain protein. Immunogenetics. 2001;53:64-8 pubmed
  12. Sackstein R, Roos M, Demant P, Colten H. Subdivision of the S region of the mouse major histocompatibility complex by identification of genomic polymorphisms of the class III genes. Immunogenetics. 1984;20:321-30 pubmed
    The S region of the mouse major histocompatibility complex (MHC) encodes the class III proteins, the second (C2) and fourth (C4) components of complement, and factor B...
  13. Atkinson J, Karp D, Seeskin E, Killion C, Rosa P, Newell S, et al. H-2 S region determined polymorphic variants of the C4, Slp, C2, and B complement proteins: a compilation. Immunogenetics. 1982;16:617-23 pubmed
  14. Seeger A, Mayer W, Klein J. A complement factor B-like cDNA clone from the zebrafish (Brachydanio rerio). Mol Immunol. 1996;33:511-20 pubmed
    ..Factor B and the complement component C2 are thought to have arisen by gene duplication...
  15. . Nomenclature for human complement component C2. WHO-IUIS Nomenclature Sub-Committee. Bull World Health Organ. 1992;70:527-30 pubmed
    This note describes the designations for variants of the human complement component C2, which were approved by the Nomenclature Committee of the International Union of Immunological Societies (IUIS).
  16. Kjalke M, Welinder K, Koch C. Structural analysis of chicken factor B-like protease and comparison with mammalian complement proteins factor B and C2. J Immunol. 1993;151:4147-52 pubmed
    ..chicken B-like protease was cleaved at a site homologous to that cleaved in mammalian complement components B and C2 on activation. Limited tryptic digestion of the B-like protease generated fragments similar to Ba and Bb...
  17. Kohchi C, Noguchi K, Tanabe Y, Mizuno D, Soma G. Constitutive expression of TNF-alpha and -beta genes in mouse embryo: roles of cytokines as regulator and effector on development. Int J Biochem. 1994;26:111-9 pubmed
    ..3. The mRNAs of several complement components (C2, C3, C4, C5) and receptors (CR1, CR2) were also detected. Among them, the expression of C3 and CR1 were prominent...
  18. Subramanian S, Yim Y, Liu K, Tus K, Zhou X, Wakeland E. Epistatic suppression of systemic lupus erythematosus: fine mapping of Sles1 to less than 1 mb. J Immunol. 2005;175:1062-72 pubmed
    ..Sle1 Sles1)F(1)s. These findings localize and characterize the suppressive properties of Sles1 and implicate 129 as a useful strain for aiding in the identification of this elusive epistatic modifier gene. ..
  19. Held K, Thiel S, Loos M, Petry F. Increased susceptibility of complement factor B/C2 double knockout mice and mannan-binding lectin knockout mice to systemic infection with Candida albicans. Mol Immunol. 2008;45:3934-41 pubmed publisher
    ..We investigated the susceptibility of mice deficient in complement factor B and C2 (Bf/C2-/-), C1q (C1qa-/-), and mannan-binding lectin (MBL)-A (MBL-A) and MBL-C (MBL-A/C-/-) to systemic infection ..
  20. Tong H, Li Y, Stahl G, Thurman J. Enhanced susceptibility to acute pneumococcal otitis media in mice deficient in complement C1qa, factor B, and factor B/C2. Infect Immun. 2010;78:976-83 pubmed publisher
    ..in acute otitis media (AOM), we investigated the susceptibility to AOM in mice deficient in complement factor B and C2 (Bf/C2(-/)(-)), C1qa (C1qa(-/)(-)), and factor B (Bf(-)(/)(-)). Bacterial titers of both S...
  21. Moreira A, Wollerton M, Monks J, Proudfoot N. Upstream sequence elements enhance poly(A) site efficiency of the C2 complement gene and are phylogenetically conserved. EMBO J. 1995;14:3809-19 pubmed
    ..We describe the first non-viral example of such an upstream sequence element (USE) for the poly(A) site of the human C2 complement gene...