Genomes and Genes
Gene Symbol: Bf
Description: complement factor B
Alias: Da1-24, complement factor B, B-factor, properdin, properdin factor B
- Senaldi G, Lupoli S, Vergani D, Black C. Activation of the complement system in systemic sclerosis. Relationship to clinical severity. Arthritis Rheum. 1989;32:1262-7 pubmed..Complement activation may therefore have a pathogenetic role in SSc, and its measurement may prove useful in monitoring the disease. ..
- Sehgal V, Sharma V, Sharma V. Comprehensive evaluation of complement components in the course of type I (Lepra) and type II (ENL) reactions. Int J Dermatol. 1989;28:32-5 pubmed..Complement components, of both classic and alternate pathways, showed no significant alterations either during type I (Lepra) reaction or after its amelioration. ..
- Doustjalali S, Yusof R, Yip C, Looi L, Pillay B, Hashim O. Aberrant expression of acute-phase reactant proteins in sera and breast lesions of patients with malignant and benign breast tumors. Electrophoresis. 2004;25:2392-401 pubmed..The relative expression of alpha1-antichymotrypsin (ACT), clusterin, and complement factor B was significantly higher in all BC patients as compared to normal controls...
- Kumar N, Kaur G, Tandon N, Mehra N. Allotyping human complement factor B in Asian Indian type 1 diabetic patients. Tissue Antigens. 2008;72:517-24 pubmed publisherHuman complement factor B (BF) is an essential component of the alternate complement pathway and therefore important in innate immune and autoimmune responses...
- Daha M, van Es L. Isolation and characterization of rat complement factor B and its interaction with cell-bound human C3. Immunology. 1980;41:849-55 pubmed..Native Brat has a beta electrophoretic mobility. The plasma concentration of Brat in Wistar rats was 215 +/- 38 microgram/ml (mean +/- SD). ..
- Nakata I, Yamashiro K, Yamada R, Gotoh N, Nakanishi H, Hayashi H, et al. Significance of C2/CFB variants in age-related macular degeneration and polypoidal choroidal vasculopathy in a Japanese population. Invest Ophthalmol Vis Sci. 2012;53:794-8 pubmed publisher..typical AMD: P = 0.0073, odds ratio [OR] = 0.47; vs. PCV: P = 0.0083, OR = 0.53). C2/CFB variants play a protective role in the risk of developing neovascular AMD and PCV in the Japanese. ..
- Dewald G, Lange C, Schmeel E, Kreysel H. HLA-linked complement polymorphisms (C2, BF) in psoriasis. Arch Dermatol Res. 1983;275:301-4 pubmed..three complement components, the authors have looked for an association between psoriasis and allotypes of C2 and BF. C2 and BF polymorphism were examined in 230 psoriatic patients...
- 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...
- Lehner T, Adinolfi M. Acute phase proteins, C9, factor B, and lysozyme in recurrent oral ulceration and Behçet's syndrome. J Clin Pathol. 1980;33:269-75 pubmed..It is suggested that the changes in the concentrations of some plasma proteins may help our understanding of tissue involvement in Behçet's syndrome, as well as in the selection of therapeutic agents in this disease. ..
- Pei X, Li X, Bao Y, Yu W, Yan Z, Qi H, et al. Association of c3 gene polymorphisms with neovascular age-related macular degeneration in a chinese population. Curr Eye Res. 2009;34:615-22 pubmed..polymorphisms (SNPs) in the C3 gene, one in the complement factor H (CFH) gene and two in the complement factor B (CFB) gene were characterized. Their genotypes, allele frequencies, and odds ratios were analyzed...
- Thurman J, Tchepeleva S, Haas M, Panzer S, Boackle S, Glogowska M, et al. Complement alternative pathway activation in the autologous phase of nephrotoxic serum nephritis. Am J Physiol Renal Physiol. 2012;302:F1529-36 pubmed publisher..Thus, although the glomerulus is resistant to antibody-initiated, alternative pathway-mediated injury, inhibition of this complement pathway may be beneficial in chronic immune complex-mediated diseases. ..
- Ferrara D, Merriam J, Freund K, Spaide R, Takahashi B, Zhitomirsky I, et al. Analysis of major alleles associated with age-related macular degeneration in patients with multifocal choroiditis: strong association with complement factor H. Arch Ophthalmol. 2008;126:1562-6 pubmed publisher..and LOC387715/HTRA1 gene on 10q26 (A69S) and protective alleles in CFH (IVS1, IVS6, and delCFHR1-3) and complement factor B loci (H9L and R32Q)...
- 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...
- Sinniah D, Yadav M. Elevated IgG and decreased complement component C3 and factor B in B-thalassaemia major. Acta Paediatr Scand. 1981;70:547-50 pubmed..The alterations in immunoglobulin and complement levels represent a secondary rather than a primary immune disorder. ..
- Saha K, Sharma V, Chakrabarty A, Sehgal V. Breakdown product of factor B as an index of complement activation in lepromatous leprosy and its relation with bacillary load. Scand J Immunol. 1983;17:37-43 pubmed..A significant negative correlation was found between serum C3 and Ba levels in most lepromatous patients. Analysis of the data suggested the alternative pathway as the possible mechanism of complement activation in lepromatous leprosy. ..
- Nelson K, Zhao M, Schroeder P, Li N, Wetsel R, Diaz L, et al. Role of different pathways of the complement cascade in experimental bullous pemphigoid. J Clin Invest. 2006;116:2892-900 pubmed..These findings provide the first direct evidence to our knowledge that complement activation via the classical and alternative pathways is crucial in subepidermal blister formation in experimental BP. ..
- Passwell J, Schreiner G, Nonaka M, Beuscher H, Colten H. Local extrahepatic expression of complement genes C3, factor B, C2, and C4 is increased in murine lupus nephritis. J Clin Invest. 1988;82:1676-84 pubmed..These results suggest that in SLE local expression of complement genes plays a role in the pathogenesis of chronic glomerulonephritis and in the autoimmune arteritis of other organs. ..
- de Messias I, Mohren D. Classical and alternative complement pathway activation in paracoccidioidomycosis. J Investig Allergol Clin Immunol. 1994;4:91-5 pubmed..03). The C3, C4 and CH50 levels did not show significant variations from the normal ranges. Our results suggest the involvement of both complement pathways, classical and alternative, in PCM and their association with disease activity. ..
- Budowle B, Acton R, Barger B, Blackstock R, Crist W, Go R, et al. Properdin factor B and acute lymphocytic leukemia (ALL). Cancer. 1982;50:2369-71 pubmed..ALL patients were compared to 545 controls for differences in phenotype and gene frequencies at the Properdin factor B locus. In addition, 90 of the ALL patients were immune phenotyped...
- Beatty D, Ryder C, Heese H. Complement abnormalities during an epidemic of group B meningococcal infection in children. Clin Exp Immunol. 1986;64:465-70 pubmed..The significant reduction in CH50 and Factor B in the more severely affected patients suggests that activation of both classical and alternate pathways occurs in group B meningococcal infection. ..
- Richardson A, Islam F, Guymer R, Baird P. Analysis of rare variants in the complement component 2 (C2) and factor B (BF) genes refine association for age-related macular degeneration (AMD). Invest Ophthalmol Vis Sci. 2009;50:540-3 pubmed publisherSeveral single-nucleotide polymorphisms (SNPs) in the C2 and BF genes have been associated with age-related macular degeneration (AMD) in Caucasian populations from the United States...
- Wyatt R, Wang C, Hudson E, Jones R, Noah P, Rosenberg E. Bf and C4 phenotypes in patients with psoriasis. Acta Derm Venereol Suppl (Stockh). 1989;146:211-3 pubmedPhenotype frequencies for the complement proteins Bf (factor B), C4A and C4B were performed in a sample of 49 Caucasian patients with psoriasis followed in Memphis, Tennessee...
- 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)...
- Wurst W, Rothermel E, Gunther E. Genetic mapping of C4 and Bf complement genes in the rat major histocompatibility complex. Immunogenetics. 1988;28:57-60 pubmed
- Anthony R, Morrison L, MacSween R, Whaley K. Biosynthesis of complement components by cultured rat hepatocytes. Biochem J. 1985;232:93-8 pubmed..It is concluded that culture of rat hepatocytes in vitro provides a useful technique for studies of the synthesis of complement components. ..
- Herrero Herrero J, Ruiz Beltrán R, Batlle Forondona J. The complement system in Mediterranean spotted fever. J Infect Dis. 1988;157:1093-5 pubmed
- Soto E, Romero R, Vaisbuch E, Erez O, Mazaki Tovi S, Kusanovic J, et al. Fragment Bb: evidence for activation of the alternative pathway of the complement system in pregnant women with acute pyelonephritis. J Matern Fetal Neonatal Med. 2010;23:1085-90 pubmed publisher..5 vs. 1.3â€‰Î¼g/ml, IQR: 1.1-1.9; p=0.2). Pregnant women with acute pyelonephritis have evidence of activation of the alternative pathway of the complement system, regardless of the presence or absence of a positive blood culture. ..
- Strife C, Prada A, Clardy C, Jackson E, Forristal J. Autoantibody to complement neoantigens in membranoproliferative glomerulonephritis. J Pediatr. 1990;116:S98-102 pubmed..The results indicate that multiple autoantibody formation is common in all MPGN types. MPGN II, and possibly MPGN I, tend to form more specific autoantibodies. ..
- Nishimukai H, Nakanishi I, Kitamura H, Tamaki Y. Factor B subtypes in Japanese patients with IgA nephropathy and with idiopathic membranous nephropathy. Exp Clin Immunogenet. 1988;5:196-202 pubmedThe present paper describes a method of subtyping of properdin factor B (BF) using polyacrylamide gel isoelectric focusing followed by immunoblotting, and the distribution of BF subtypes in Japanese patients with IgA nephropathy or ..
- 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 pubmedTo 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 ..
- Anyaegbu C, Okpala I, Aken ova A, Salimonu L. Complement haemolytic activity, circulating immune complexes and the morbidity of sickle cell anaemia. APMIS. 1999;107:699-702 pubmed..cell anaemia (SCA) relate to complement function, or the levels of circulating immune complexes (CIC), complement factor B (Bf), C3 and C4...
- Bitencourt C, Duarte C, Azzolini A, Assis Pandochi A. Alternative complement pathway and factor B activities in rats with altered blood levels of thyroid hormone. Braz J Med Biol Res. 2012;45:216-21 pubmed..We conclude that alterations in thyroid hormone levels affect the activity of the AP and factor B, which may in turn affect the roles of AP and factor B in antibody production. ..
- Rambausek M, van den Wall Bake A, Schumacher Ach R, Spitzenberg R, Rother U, van Es L, et al. Genetic polymorphism of C3 and Bf in IgA nephropathy. Nephrol Dial Transplant. 1987;2:208-11 pubmedC3 and Bf alleles were examined in the general population, in 67 patients with biopsy-confirmed mesangial IgA nephropathy and 81 patients with other types of glomerulonephritis, from the Heidelberg and Leiden renal programmes respectively...
- SubÃas M, Tortajada A, Gastoldi S, Galbusera M, LÃ³pez Perrote A, Lopez L, et al. A novel antibody against human factor B that blocks formation of the C3bB proconvertase and inhibits complement activation in disease models. J Immunol. 2014;193:5567-75 pubmed publisher..As a whole, these data demonstrate the potential value of FB28.4.2 for the treatment of disorders associated with AP complement dysregulation in man and animal models. ..
- Oren R, Laufer J, Goldberg I, Kopolovic J, Waldherr R, Passwell J. C3, C4, factor B and HLA-DR alpha mRNA expression in renal biopsy specimens from patients with IgA nephropathy. Immunology. 1995;86:575-83 pubmed..Our findings, which reflect the immunopathogenic events in vivo, provide new insights as to the interpretation of the molecular immunology of this immune complex disease. ..
- Lenderink A, Liegel K, Ljubanovic D, Coleman K, Gilkeson G, Holers V, et al. The alternative pathway of complement is activated in the glomeruli and tubulointerstitium of mice with adriamycin nephropathy. Am J Physiol Renal Physiol. 2007;293:F555-64 pubmed..These results demonstrate that complement activation in this nonimmune complex-mediated model of progressive renal disease requires an intact alternative pathway. ..
- Ogunleye A, Arinola O. Immunoglobulin classes, complement factors and circulating immune complexes in chronic sinusitis patients. Afr J Med Med Sci. 2001;30:309-12 pubmedSerum concentrations of immunoglobulin classes (IgG, IgA and IgM), complement factor B (FB), 4th complement factor (C4) and circulating immune complexes (CIC) were determined in 34 Nigerians with chronic sinusitis and 37 apparently ..
- 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...
- Villaescusa Blanco R, Arce A, Santos R, Fernandez N. [Changes of the complement system in myelodysplastic syndromes]. Sangre (Barc). 1998;43:210-2 pubmed..Our results suggest the possibility of subclinical infections in patients with unfavourable prognosis. ..
- Buentello Volante B, Rodriguez Ruiz G, Miranda Duarte A, Pompa Mera E, Graue Wiechers F, Bekker Mendez C, et al. Susceptibility to advanced age-related macular degeneration and alleles of complement factor H, complement factor B, complement component 2, complement component 3, and age-related maculopathy susceptibility 2 genes in a Mexican population. Mol Vis. 2012;18:2518-25 pubmed..association of age-related macular degeneration (AMD)-high risk alleles of the complement factor H (CFH), complement factor B (CFB), complement component 2 (C2), complement component 3 (C3), and age-related maculopathy susceptibility ..
- Sundsmo J, Papin R, Wood L, Hirani S, Waldeck N, Buckingham B, et al. Complement activation in type 1 human diabetes. Clin Immunol Immunopathol. 1985;35:211-25 pubmed..abstract truncated at 400 words) ..
- Goicoechea de Jorge E, Harris C, Esparza Gordillo J, Carreras L, Arranz E, Garrido C, et al. Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc Natl Acad Sci U S A. 2007;104:240-5 pubmed..alternative pathway and found within this subgroup two families with mutations in the gene encoding factor B (BF), a zymogen that carries the catalytic site of the complement alternative pathway convertase (C3bBb)...
- Oppermann M, Kurts C, Zierz R, Quentin E, Weber M, Gotze O. Elevated plasma levels of the immunosuppressive complement fragment Ba in renal failure. Kidney Int. 1991;40:939-47 pubmed..These results suggest that the accumulation of Ba contributes to the defective immune response in patients with renal failure. ..
- Wu L, Tao Q, Chen W, Wang Z, Song Y, Sheng S, et al. Association between polymorphisms of complement pathway genes and age-related macular degeneration in a Chinese population. Invest Ophthalmol Vis Sci. 2013;54:170-4 pubmed publisher..genes, including rs800292 and rs1410996 of complement H (CFH), rs9332739 of complement 2 (C2), rs4151667 of complement factor B (CFB), and rs2241394 and rs2230199 of complement 3 (C3)...
- Su Z, Wang X, Gao X, Liu Y, Pan C, Hu H, et al. Excessive activation of the alternative complement pathway in autosomal dominant polycystic kidney disease. J Intern Med. 2014;276:470-85 pubmed publisher..Screening of the glycoproteome of urine samples from ADPKD patients revealed that levels of complement factor B (CFB), serpin peptidase inhibitor, complement component 1 inhibitor (SERPING1) and complement component 9 (..
- Ballow M, Donshik P, Mendelson L. Complement proteins and C3 anaphylatoxin in the tears of patients with conjunctivitis. J Allergy Clin Immunol. 1985;76:473-6 pubmed
- Haglund L, Bernier S, Onnerfjord P, Recklies A. Proteomic analysis of the LPS-induced stress response in rat chondrocytes reveals induction of innate immune response components in articular cartilage. Matrix Biol. 2008;27:107-18 pubmed..traditionally associated with the innate immune response, such as the complement components C1r, C3 and complement factor B, long pentraxin-3 and osteoglycin...
- Mondino B, Glovsky M, Ghekiere L. Activated complement in inflamed aqueous humor. Invest Ophthalmol Vis Sci. 1984;25:871-3 pubmed..Activated complement fragments are present in the aqueous humor of eyes with anterior uveitis and may help mediate the inflammatory process. ..
- Chen M, Muckersie E, Luo C, Forrester J, Xu H. Inhibition of the alternative pathway of complement activation reduces inflammation in experimental autoimmune uveoretinitis. Eur J Immunol. 2010;40:2870-81 pubmed publisherWe have shown previously that complement factor H (CFH) and complement factor B (CFB) are constitutively expressed by retinal pigment epithelial cells and their production is regulated by inflammatory cytokines, suggesting that the ..
- Verroust F, Adam C, Kourilsky O, Allain J, Verroust P. Circulating immune complexes and complement levels in hemophilic children. J Clin Lab Immunol. 1981;6:127-30 pubmed..Levels of the C3 breakdown product C3d were significantly raised suggesting intravascular complement activation. The significance of these abnormalities is discussed in relation to perfusion of Factor VIII preparations. ..
- 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...
- 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...
- Sassi F, Bardi R, Neji T, Ayed K, Ben Dridi M. [Immunological study in sickle cell disease patients: importance of the complement system]. Tunis Med. 2003;81:195-9 pubmed..Numeration of Band T cells revealed slight decrease in proportion of CD3 and CD4 at one patient associated with an increase in B cells, but normal or increased absolute numbers of all cells population. ..
- Thurman J, Royer P, Ljubanovic D, Dursun B, Lenderink A, Edelstein C, et al. Treatment with an inhibitory monoclonal antibody to mouse factor B protects mice from induction of apoptosis and renal ischemia/reperfusion injury. J Am Soc Nephrol. 2006;17:707-15 pubmed..Thus, use of an inhibitory mAb to mouse factor B effectively prevented activation of complement in the kidney after I/R and protected the mice from necrotic and apoptotic injury of the tubules. ..