Gene Symbol: Cfb
Description: complement factor B
Alias: AI195813, AI255840, H2-Bf, complement factor B, C3/C5 convertase, Factor B, alternative-complement pathway C3/C5 convertase, complement component 2 (within H-2S), histocompatibility 2, complement component factor B, properdin
Species: mouse
Products:     Cfb

Top Publications

  1. Maga T, Nishimura C, Weaver A, Frees K, Smith R. Mutations in alternative pathway complement proteins in American patients with atypical hemolytic uremic syndrome. Hum Mutat. 2010;31:E1445-60 pubmed publisher
    ..complement factor H (CFH), complement factor H-related 5 (CFHR5), complement factor I (CFI), CD46 (MCP), complement factor B (CFB), complement component 3 (C3) and thrombomodulin (THBD)...
  2. Tu Z, Bu H, Dennis J, Lin F. Efficient osteoclast differentiation requires local complement activation. Blood. 2010;116:4456-63 pubmed publisher
    ..In addition to C3 receptors, C3aR/C5aR also regulate OC differentiation, at least in part, by modulating local IL-6 production. ..
  3. Wu X, Spitzer D, Mao D, Peng S, Molina H, Atkinson J. Membrane protein Crry maintains homeostasis of the complement system. J Immunol. 2008;181:2732-40 pubmed
    ..In this study, we demonstrate that Crry(-/-) mice could be rescued on a partial as well as on a complete factor B (fB)- or C3-deficient maternal background...
  4. 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
    b>Factor B is a serine protease, essential for the function of the alternative pathway of complement activation...
  5. Walsh M, Bourcier T, Takahashi K, Shi L, Busche M, Rother R, et al. Mannose-binding lectin is a regulator of inflammation that accompanies myocardial ischemia and reperfusion injury. J Immunol. 2005;175:541-6 pubmed
    ..MBL is an example of a pattern recognition molecule that plays a dual role in modifying inflammatory responses to sterile and infectious injury. ..
  6. Hart M, Ceonzo K, Shaffer L, Takahashi K, Rother R, Reenstra W, et al. Gastrointestinal ischemia-reperfusion injury is lectin complement pathway dependent without involving C1q. J Immunol. 2005;174:6373-80 pubmed GI/R injury was evaluated using C1q-deficient (C1q KO), MBL-A/C-deficient (MBL-null), complement factor 2- and factor B-deficient (C2/fB KO), and wild-type (WT) mice...
  7. Kapadia S, Levine B, Speck S, Virgin H. Critical role of complement and viral evasion of complement in acute, persistent, and latent gamma-herpesvirus infection. Immunity. 2002;17:143-55 pubmed
  8. Matsumoto M, Fukuda W, Circolo A, Goellner J, Strauss Schoenberger J, Wang X, et al. Abrogation of the alternative complement pathway by targeted deletion of murine factor B. Proc Natl Acad Sci U S A. 1997;94:8720-5 pubmed
    To investigate the role of complement protein factor B (Bf) and alternative pathway activity in vivo, and to test the hypothesized potential genetic lethal effect of Bf deficiency, the murine Bf gene was interrupted by exchange of exon 3 ..
  9. Nataf S, Carroll S, Wetsel R, Szalai A, Barnum S. Attenuation of experimental autoimmune demyelination in complement-deficient mice. J Immunol. 2000;165:5867-73 pubmed
    ..In this report, we used mice deficient in either C3 or factor B to clarify the role of the complement system in an Ab-independent model of EAE...

More Information


  1. 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
    ..We found that S. pneumoniae induced increased gene expression of factor B of the alternative complement pathway and C3 in mouse middle ear epithelium...
  2. Schwaeble W, Lynch N, Clark J, Marber M, Samani N, Ali Y, et al. 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. 2011;108:7523-8 pubmed publisher
    ..The therapeutic effects of MASP-2 inhibition in this experimental model suggest the utility of anti-MASP-2 antibody therapy in reperfusion injury and other lectin pathway-mediated disorders. ..
  3. Banda N, Takahashi M, Takahashi K, Stahl G, Hyatt S, Glogowska M, et al. Mechanisms of mannose-binding lectin-associated serine proteases-1/3 activation of the alternative pathway of complement. Mol Immunol. 2011;49:281-9 pubmed publisher
    ..We conclude that MASP-1 does not require binding to MBL-A, MBL-C, or FCN-A to activate the AP. MASP-1 may cleave pro-Df into mature Df through binding to FCN-B or to an unknown protein, or may function as an unbound soluble protein. ..
  4. 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
    ..pneumoniae 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...
  5. Rohrer B, Long Q, Coughlin B, Wilson R, Huang Y, Qiao F, et al. A targeted inhibitor of the alternative complement pathway reduces angiogenesis in a mouse model of age-related macular degeneration. Invest Ophthalmol Vis Sci. 2009;50:3056-64 pubmed publisher
    ..CR2-fH was effective in reducing CNV and provided approximately 60% of the amount of protection of that seen in factor B-deficient mice that lacked functional AP...
  6. Thurman J, Ljubanovic D, Edelstein C, Gilkeson G, Holers V. Lack of a functional alternative complement pathway ameliorates ischemic acute renal failure in mice. J Immunol. 2003;170:1517-23 pubmed
    ..that complement activation in this setting occurs via the alternative pathway and that mice deficient in complement factor B, an essential component of the alternative pathway, would be protected from ischemic ARF...
  7. Zou L, Feng Y, Li Y, Zhang M, Chen C, Cai J, et al. Complement factor B is the downstream effector of TLRs and plays an important role in a mouse model of severe sepsis. J Immunol. 2013;191:5625-35 pubmed publisher
    ..In this article, we show that activation of TLR2, TLR3, and TLR4 markedly enhanced complement factor B (cfB) synthesis and release by macrophages and cardiac cells...
  8. Banda N, Thurman J, Kraus D, Wood A, Carroll M, Arend W, et al. Alternative complement pathway activation is essential for inflammation and joint destruction in the passive transfer model of collagen-induced arthritis. J Immunol. 2006;177:1904-12 pubmed
    ..C57BL/6 mice were used that were genetically deficient in either the alternative pathway protein factor B (Bf(-/-)) or in the classical pathway component C4 (C4(-/-))...
  9. Mehlhop E, Diamond M. Protective immune responses against West Nile virus are primed by distinct complement activation pathways. J Exp Med. 2006;203:1371-81 pubmed
    ..Genetic deficiencies in C1q, C4, factor B, or factor D all resulted in increased mortality in mice, suggesting that all activation pathways function ..
  10. Woodell A, Jones B, Williamson T, Schnabolk G, Tomlinson S, Atkinson C, et al. A Targeted Inhibitor of the Alternative Complement Pathway Accelerates Recovery From Smoke-Induced Ocular Injury. Invest Ophthalmol Vis Sci. 2016;57:1728-37 pubmed publisher
    ..Improving our understanding of the regulation of the AP is paramount to developing novel treatment approaches for AMD. ..
  11. 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
    ..The distances between the E alpha and 21-OHB genes is 430 kb and between the C2 and TNF-alpha genes at least 420 kb. ..
  12. Ma W, Coon S, Zhao L, Fariss R, Wong W. A2E accumulation influences retinal microglial activation and complement regulation. Neurobiol Aging. 2013;34:943-60 pubmed publisher
    ..Significantly, A2E accumulation altered microglial complement regulation by increasing complement factor B and decreasing complement factor H expression, favoring increased complement activation and deposition in ..
  13. Thurman J, Lenderink A, Royer P, Coleman K, Zhou J, Lambris J, et al. C3a is required for the production of CXC chemokines by tubular epithelial cells after renal ishemia/reperfusion. J Immunol. 2007;178:1819-28 pubmed
    ..This innate immune system thereby recognizes hypoxic injury and triggers a systemic inflammatory response through the generation of C3a and subsequent activation of the NF-kappaB system. ..
  14. Persson L, Boren J, Robertson A, Wallenius V, Hansson G, Pekna M. Lack of complement factor C3, but not factor B, increases hyperlipidemia and atherosclerosis in apolipoprotein E-/- low-density lipoprotein receptor-/- mice. Arterioscler Thromb Vasc Biol. 2004;24:1062-7 pubmed
    ..deficiency on atherogenesis and lipidemia, we used mice deficient in the third complement component (C3-/-) or factor B (FB-/-)...
  15. Bora N, Kaliappan S, Jha P, Xu Q, Sohn J, Dhaulakhandi D, et al. Complement activation via alternative pathway is critical in the development of laser-induced choroidal neovascularization: role of factor B and factor H. J Immunol. 2006;177:1872-8 pubmed
    ..and alternative pathways were blocked in C57BL/6 mice by small interfering RNAs (siRNA) directed against C1q and factor B, respectively...
  16. 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. ..
  17. 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
    ..To explore 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...
  18. Kaczorowski D, Afrazi A, Scott M, Kwak J, Gill R, Edmonds R, et al. Pivotal advance: The pattern recognition receptor ligands lipopolysaccharide and polyinosine-polycytidylic acid stimulate factor B synthesis by the macrophage through distinct but overlapping mechanisms. J Leukoc Biol. 2010;88:609-18 pubmed publisher
    ..Using a RT-PCR-based panel, we demonstrate that of 18 complement components tested, factor B of the alternative pathway is the most robustly up-regulated complement component in macrophages in response to ..
  19. Womack J, Hawes N, Soares E, Roderick T. Mitochondrial malate dehydrogenase (Mor-1) in the mouse: linkage to chromosome 5 markers. Biochem Genet. 1975;13:519-25 pubmed
    ..Thus Mor-1 is presently the most distal marker on chromosome 5. Three different nuclear loci for mitochondrial enzymes (Mod-2, Got-2, and Mor-1) have now been mapped in the mouse, all on different chromosomes. ..
  20. Schlagel C, Ahmed A. Evidence for genetic control of microwave-induced augmentation of complement receptor-bearing B lymphocytes. J Immunol. 1982;129:1530-3 pubmed
    ..The C57BL/6JTy-le strain remained nonresponsive. This places the essential regulatory gene to the right of the PgM-1 locus and to the left of the rd locus on chromosome 5. ..
  21. Hietala M, Nandakumar K, Persson L, Fahlén S, Holmdahl R, Pekna M. Complement activation by both classical and alternative pathways is critical for the effector phase of arthritis. Eur J Immunol. 2004;34:1208-16 pubmed
    ..pathways of complement activation in the effector phase of arthritis, we have induced arthritis in C3- and factor B (FB)-deficient (C3(-/-) and FB(-/-)) DBA/1J mice using well-defined monoclonal IgG2b and IgG2a antibodies to type ..
  22. Saeland E, Vidarsson G, Leusen J, Van Garderen E, Nahm M, Vile Weekhout H, et al. Central role of complement in passive protection by human IgG1 and IgG2 anti-pneumococcal antibodies in mice. J Immunol. 2003;170:6158-64 pubmed
    ..C1q or C2/factor B knockout mice, however, were not protected by passive immunization...
  23. Sobek Klocke I, Disqué Kochem C, Ronsiek M, Klocke R, Jockusch H, Breuning A, et al. The human gene ZFP161 on 18p11.21-pter encodes a putative c-myc repressor and is homologous to murine Zfp161 (Chr 17) and Zfp161-rs1 (X Chr). Genomics. 1997;43:156-64 pubmed
    ..Mapping of Zfp161 confirms and extends a region of homology between distal mouse chromosome 17 and human 18p. ..
  24. Hu X, Holers V, Thurman J, Schoeb T, Ramos T, Barnum S. Therapeutic inhibition of the alternative complement pathway attenuates chronic EAE. Mol Immunol. 2013;54:302-8 pubmed publisher
    ..Using a well-characterized inhibitory monoclonal antibody (mAb 1379) directed against mouse factor B, we assessed the therapeutic value of inhibiting the alternative complement pathway in experimental autoimmune ..
  25. Peng Q, Li K, Anderson K, Farrar C, Lu B, Smith R, et al. Local production and activation of complement up-regulates the allostimulatory function of dendritic cells through C3a-C3aR interaction. Blood. 2008;111:2452-61 pubmed
    ..In addition, DCs lacking factor B were unable to generate potent T-cell responses against donor antigen, whereas lack of C4 had no detectable ..
  26. Sweigard J, Yanai R, Gaissert P, Saint Geniez M, Kataoka K, Thanos A, et al. The alternative complement pathway regulates pathological angiogenesis in the retina. FASEB J. 2014;28:3171-82 pubmed publisher
    ..Sweigard, J. H., Yanai, R., Gaissert, P., Saint-Geniez, M., Kataoka, K., Thanos, A., Stahl, G. L., Lambris, J. D., Connor, K. M. The alternative complement pathway regulates pathological angiogenesis in the retina. ..
  27. Tong H, Lambert G, Li Y, Thurman J, Stahl G, Douthitt K, et al. Deletion of the complement C5a receptor alleviates the severity of acute pneumococcal otitis media following influenza A virus infection in mice. PLoS ONE. 2014;9:e95160 pubmed publisher
    ..Mice deficient in complement C1qa (C1qa-/-) or factor B (Bf -/-) exhibited delayed viral and bacterial clearance from the middle ear and developed significant mucosal ..
  28. Singh M, Kapoun A, Higgins L, Kutschke W, Thurman J, Zhang R, et al. Ca2+/calmodulin-dependent kinase II triggers cell membrane injury by inducing complement factor B gene expression in the mouse heart. J Clin Invest. 2009;119:986-96 pubmed publisher
    ..We studied 1 of these proinflammatory genes, complement factor B (Cfb), in detail, because complement proteins secreted by cells other than cardiomyocytes can induce ..
  29. 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
    ..complex (MHC) encodes the class III proteins, the second (C2) and fourth (C4) components of complement, and factor B. Previously, the assignment of S-region haplotypes was based on analysis of protein polymorphisms...
  30. Chaplin D, Woods D, Whitehead A, Goldberger G, Colten H, Seidman J. Molecular map of the murine S region. Proc Natl Acad Sci U S A. 1983;80:6947-51 pubmed
    Eighteen overlapping cosmid clones spanning 240 kilobases and encoding the gene for factor B and two genes related to the fourth component of complement (C4) were isolated from a murine H-2d genomic library...
  31. Lee H, Green D, Lai L, Hou Y, Jensenius J, Liu D, et al. Early complement factors in the local tissue immunocomplex generated during intestinal ischemia/reperfusion injury. Mol Immunol. 2010;47:972-81 pubmed publisher
    ..In addition, C1q, the initial molecule of the classical pathway was also detected on the immunocomplex. However, Factor B, the early molecule in the alternative pathway, was not detected in the immunocomplex...
  32. Ji H, Ohmura K, Mahmood U, Lee D, Hofhuis F, Boackle S, et al. Arthritis critically dependent on innate immune system players. Immunity. 2002;16:157-68 pubmed
    ..We suggest that autoimmune disease, even one that is organ specific, can occur when mobilization of an adaptive immune response results in runaway activation of the innate response. ..
  33. Tchepeleva S, Thurman J, Ruff K, Perkins S, Morel L, Boackle S. An allelic variant of Crry in the murine Sle1c lupus susceptibility interval is not impaired in its ability to regulate complement activation. J Immunol. 2010;185:2331-9 pubmed publisher
  34. 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...
  35. Markiewski M, DeAngelis R, Benencia F, Ricklin Lichtsteiner S, Koutoulaki A, Gerard C, et al. Modulation of the antitumor immune response by complement. Nat Immunol. 2008;9:1225-35 pubmed publisher
    ..Thus, our study demonstrates a therapeutic function for complement inhibition in the treatment of cancer. ..
  36. 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
    b>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...
  37. Gunn B, Morrison T, Whitmore A, Blevins L, Hueston L, Fraser R, et al. Mannose binding lectin is required for alphavirus-induced arthritis/myositis. PLoS Pathog. 2012;8:e1002586 pubmed publisher
  38. Girardi G, Berman J, Redecha P, Spruce L, Thurman J, Kraus D, et al. Complement C5a receptors and neutrophils mediate fetal injury in the antiphospholipid syndrome. J Clin Invest. 2003;112:1644-54 pubmed
    ..Studies in factor B-deficient mice, however, indicate that alternative pathway activation is required and amplifies complement ..
  39. Lee H, Wysoczynski M, Liu R, Shin D, Kucia M, Botto M, et al. Mobilization studies in complement-deficient mice reveal that optimal AMD3100 mobilization of hematopoietic stem cells depends on complement cascade activation by AMD3100-stimulated granulocytes. Leukemia. 2010;24:573-82 pubmed publisher
    ..b>Cfb(-/-)) as well as in mice that do not activate the distal steps of CC (C5(-/-))...
  40. Pavlov V, La Bonte L, Baldwin W, Markiewski M, Lambris J, Stahl G. Absence of mannose-binding lectin prevents hyperglycemic cardiovascular complications. Am J Pathol. 2012;180:104-12 pubmed publisher
    ..Together, these data suggest that MBL and the lectin complement pathway play a significant role in vascular dysfunction and cardiomyopathy after acute hyperglycemia. ..
  41. Pekna M, Hietala M, Landin A, Nilsson A, Lagerberg C, Betsholtz C, et al. Mice deficient for the complement factor B develop and reproduce normally. Scand J Immunol. 1998;47:375-80 pubmed
    b>Factor B is an essential component of the complement cascade which forms the C3 and C5 convertase of the alternative pathway...
  42. 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
    ..Unexpectedly, we observed that C4(-/-) and factor B(-/-) mice were fully susceptible to disease, indicating that activation of the classical or alternative pathways ..
  43. Schnabolk G, Coughlin B, Joseph K, Kunchithapautham K, Bandyopadhyay M, O Quinn E, et al. Local production of the alternative pathway component factor B is sufficient to promote laser-induced choroidal neovascularization. Invest Ophthalmol Vis Sci. 2015;56:1850-63 pubmed publisher
    b>Complement factor B (CFB) is a required component of the alternative pathway (AP) of complement, and CFB polymorphisms are associated with age-related macular degeneration (AMD) risk...
  44. Huang Y, Krein P, Muruve D, Winston B. Complement factor B gene regulation: synergistic effects of TNF-alpha and IFN-gamma in macrophages. J Immunol. 2002;169:2627-35 pubmed
    b>Complement factor B (Bf) plays an important role in activating the alternative complement pathway. The inflammatory cytokines, in particular TNF-alpha and IFN-gamma, are critical in the regulation of Bf gene expression in macrophages...
  45. Leinhase I, Holers V, Thurman J, Harhausen D, Schmidt O, Pietzcker M, et al. Reduced neuronal cell death after experimental brain injury in mice lacking a functional alternative pathway of complement activation. BMC Neurosci. 2006;7:55 pubmed contributing to neuronal cell death, based on a standardized TBI model in mice with targeted deletion of the factor B gene (fB-/-), a "key" component required for activation of the alternative complement pathway...
  46. Elvington A, Atkinson C, Zhu H, Yu J, Takahashi K, Stahl G, et al. The alternative complement pathway propagates inflammation and injury in murine ischemic stroke. J Immunol. 2012;189:4640-7 pubmed publisher
    ..In this study, we show that compared with wild-type mice, mice deficient in the alternative pathway protein factor B or mice treated with the alternative pathway inhibitor CR2-fH have improved outcomes after 60-min middle cerebral ..
  47. Roos M, Demant P. Murine complement factor B (BF): sexual dimorphism and H-2-linked polymorphism. Immunogenetics. 1982;15:23-30 pubmed
    ..These data extend the information on the interspecies homology of the MHC and may open new possibilities for studies of the genetic organization and hormonal regulation of the H-2 complex. ..
  48. Clark A, Weymann A, Hartman E, Turmelle Y, Carroll M, Thurman J, et al. Evidence for non-traditional activation of complement factor C3 during murine liver regeneration. Mol Immunol. 2008;45:3125-32 pubmed publisher
    ..we investigated the hepatic regenerative response to partial hepatectomy in wildtype mice, C3-, C4-, and factor B-null mice, and C4-null mice treated with a factor B neutralizing antibody (mAb 1379)...
  49. Watanabe H, Garnier G, Circolo A, Wetsel R, Ruiz P, Holers V, et al. Modulation of renal disease in MRL/lpr mice genetically deficient in the alternative complement pathway factor B. J Immunol. 2000;164:786-94 pubmed
    ..To determine the role of the alternative pathway in lupus nephritis, complement factor B-deficient mice were backcrossed to MRL/lpr mice...
  50. Malik T, Cortini A, Carassiti D, Boyle J, Haskard D, Botto M. The alternative pathway is critical for pathogenic complement activation in endotoxin- and diet-induced atherosclerosis in low-density lipoprotein receptor-deficient mice. Circulation. 2010;122:1948-56 pubmed publisher
    ..Mice lacking factor B (Bf(-/-)), the initiator of the alternative pathway, were crossed with Ldlr(-/-) mice and studied under different ..
  51. Renner B, Coleman K, Goldberg R, Amura C, Holland Neidermyer A, Pierce K, et al. The complement inhibitors Crry and factor H are critical for preventing autologous complement activation on renal tubular epithelial cells. J Immunol. 2010;185:3086-94 pubmed publisher
    ..Finally, we injected Crry(-/-)fB(-/-) and Crry(+/+)fB(-/-) mice with purified factor B (an essential protein of the alternative pathway)...
  52. Paglialunga S, Fisette A, Yan Y, Deshaies Y, Brouillette J, Pekna M, et al. Acylation-stimulating protein deficiency and altered adipose tissue in alternative complement pathway knockout mice. Am J Physiol Endocrinol Metab. 2008;294:E521-9 pubmed
    ..Mice lacking alternative complement factor B or adipsin (FBKO or ADKO), required for ASP production, were also ASP deficient...
  53. Wang J, Ohno Matsui K, Yoshida T, Shimada N, Ichinose S, Sato T, et al. Amyloid-beta up-regulates complement factor B in retinal pigment epithelial cells through cytokines released from recruited macrophages/microglia: Another mechanism of complement activation in age-related macular degeneration. J Cell Physiol. 2009;220:119-28 pubmed publisher
    ..The purpose of this study was to investigate the influence of Abeta on factor B, the main activator of the complement alternative pathway...
  54. Miwa T, Sato S, Gullipalli D, Nangaku M, Song W. Blocking properdin, the alternative pathway, and anaphylatoxin receptors ameliorates renal ischemia-reperfusion injury in decay-accelerating factor and CD59 double-knockout mice. J Immunol. 2013;190:3552-9 pubmed publisher
    ..mice with mice deficient in various complement components or receptors including C3, C4, factor B (fB), factor properdin (fP), mannose-binding lectin, C3aR, C5aR, or Ig and assessed renal IRI in the resulting mutant strains...
  55. Hietala M, Jonsson I, Tarkowski A, Kleinau S, Pekna M. Complement deficiency ameliorates collagen-induced arthritis in mice. J Immunol. 2002;169:454-9 pubmed
    ..DBA/1J mice deficient of complement factors C3 (C3(-/-)) and factor B (FB(-/-)) were generated to elucidate the role of the complement system in CIA...
  56. Hutchinson W, Herbert J, Botto M, Pepys M. Classical and alternative pathway complement activation are not required for reactive systemic AA amyloid deposition in mice. Immunology. 2004;112:250-4 pubmed
    ..More importantly, mice with targeted deletion of the genes for C1q or for both factor B and C2, and therefore unable to sustain activation, respectively, of either the classical complement pathway or ..
  57. 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
    An important molecule in the activation of the complement system in vertebrates is factor B, a serine protease with a molecular mass of 95,000. Factor B and the complement component C2 are thought to have arisen by gene duplication...
  58. Mabbott N, Bruce M, Botto M, Walport M, Pepys M. Temporary depletion of complement component C3 or genetic deficiency of C1q significantly delays onset of scrapie. Nat Med. 2001;7:485-7 pubmed
    ..Thus, in the early stages of infection, C3 and perhaps C1q contribute to the localization of TSE infectivity in lymphoid tissue and may be therapeutic targets. ..
  59. Mueller Ortiz S, Drouin S, Wetsel R. 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. 2004;72:2899-906 pubmed
    ..aeruginosa pulmonary infection, we challenged C3-, C4-, and factor B-deficient mice with P. aeruginosa via intranasal inoculation...
  60. Rohrer B, Coughlin B, Kunchithapautham K, Long Q, Tomlinson S, Takahashi K, et al. The alternative pathway is required, but not alone sufficient, for retinal pathology in mouse laser-induced choroidal neovascularization. Mol Immunol. 2011;48:e1-8 pubmed publisher
    ..Proteins encoded by the AMD risk genes participate in the AP (CFB), CP/LP (C2), or in the AP and final common pathway (C3)...
  61. Taube C, Thurman J, Takeda K, Joetham A, Miyahara N, Carroll M, et al. Factor B of the alternative complement pathway regulates development of airway hyperresponsiveness and inflammation. Proc Natl Acad Sci U S A. 2006;103:8084-9 pubmed
    ..we studied mice deficient in complement factor 4 (C4-/-), a critical component of the classical pathway, or factor B (fB-/-), an essential protein in the alternative complement pathway...
  62. Pickering M, Cook H, Warren J, Bygrave A, Moss J, Walport M, et al. Uncontrolled C3 activation causes membranoproliferative glomerulonephritis in mice deficient in complement factor H. Nat Genet. 2002;31:424-8 pubmed
    ..Introducing a second mutation in the gene encoding complement factor B, which prevents C3 turnover in vivo, obviates the phenotype of Cfh(-/-) mice...
  63. Quartier P, Potter P, Ehrenstein M, Walport M, Botto M. 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. 2005;35:252-60 pubmed
    ..Hence, the efficient uptake of dying cells by BMDM requires IgM antibodies and complement. ..
  64. Zhou H, Yan H, Bertram P, Hu Y, Springer L, Thompson R, et al. Fibrinogen-specific antibody induces abdominal aortic aneurysm in mice through complement lectin pathway activation. Proc Natl Acad Sci U S A. 2013;110:E4335-44 pubmed publisher
    ..Our findings support the concept that an autoimmune process directed at aortic wall self-antigens may play a central role in the immunopathogenesis of AAA. ..
  65. Xiao H, Schreiber A, Heeringa P, Falk R, Jennette J. Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies. Am J Pathol. 2007;170:52-64 pubmed
    ..common pathway component C5, classic and lectin binding pathway component C4, and alternative pathway component factor B. After injection of anti-MPO IgG, C4-/- mice developed disease comparable with wild-type disease; however, C5-/- ..
  66. Banda N, Takahashi M, Levitt B, Glogowska M, Nicholas J, Takahashi K, et al. Essential role of complement mannose-binding lectin-associated serine proteases-1/3 in the murine collagen antibody-induced model of inflammatory arthritis. J Immunol. 2010;185:5598-606 pubmed publisher
  67. Garnier G, Ault B, Kramer M, Colten H. cis and trans elements differ among mouse strains with high and low extrahepatic complement factor B gene expression. J Exp Med. 1992;175:471-9 pubmed
    b>Factor B (Bf), an enzyme of the alternative pathway of complement activation, is one of four major histocompatibility complex (MHC) class III genes...
  68. Yoshino M, Sagai T, Lindahl K, Toyoda Y, Shirayoshi Y, Matsumoto K, et al. Recombination in the class III region of the mouse major histocompatibility complex. Immunogenetics. 1994;40:280-6 pubmed
    ..The result demonstrated that an unequal distribution of recombination is a general feature of the mouse MHC, suggesting the presence of a recombinational hotspot within the Int3:Tnx interval. ..
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    ..Mice deficient in the alternative pathway protein factor B (fB) were protected from traumatic SCI in terms of reduced tissue damage and demyelination, reduced inflammatory ..
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    ..Using mice and human serum deficient in either C1q, the first component of the classical pathway, or factor B, an important component of the alternative pathway, we have investigated the role of both pathways for innate ..
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    ..Splenic accumulation of prion infectivity and PrPSc was delayed, indicating that activation of specific complement components is involved in the initial trapping of prions in lymphoreticular organs early after infection. ..
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    A hemolytic assay has been developed which is specific for Factor B (B) activity in murine EDTA-plasma. Three discrete levels of B activity were observed among B10-congenic strains...
  74. Pinto A, RAMOS H, Wu X, Aggarwal S, Shrestha B, Gorman M, et al. Deficient IFN signaling by myeloid cells leads to MAVS-dependent virus-induced sepsis. PLoS Pathog. 2014;10:e1004086 pubmed publisher massive complement activation, as liver damage was minimized in animals lacking complement components C3 or factor B or treated with neutralizing anti-C5 antibodies...
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    ..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 ..
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    We demonstrate the presence of complement factor B (Bf) and complement C3 in uterine luminal fluid collected from estrogen-stimulated immature and adult female mice...
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    ..This antigen-independent mechanism for classical pathway activation may augment activation of the complement system at sites of inflammation and infarction. ..
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    ..effects of CE were analyzed in wildtype (WT) mice or mice without a functional complement alternative pathway (AP; CFB(-/-) ) using molecular, histological, electrophysiological, and behavioral outcomes...
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    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 ..
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  82. Byrne S, Hammond K, Chan C, Rogers L, Beaugie C, Rana S, et al. The alternative complement component factor B regulates UV-induced oedema, systemic suppression of contact and delayed hypersensitivity, and mast cell infiltration into the skin. Photochem Photobiol Sci. 2015;14:801-6 pubmed publisher
    ..enrichment analysis of microarray data, we identified the alternative complement pathway with a central role for factor B (fB) in UVA-induced immunosuppression...
  83. Jain U, Cao Q, Thomas N, Woodruff T, Schwaeble W, Stover C, et al. Properdin provides protection from Citrobacter rodentium-induced intestinal inflammation in a C5a/IL-6-dependent manner. J Immunol. 2015;194:3414-21 pubmed publisher
    ..In this study, we examined the impact of the lack of properdin, a positive regulator of complement, in C. rodentium-induced colitis...