This gene encodes complement factor B, a component of the alternative pathway of complement activation. Factor B circulates in the blood as a single chain polypeptide. Upon activation of the alternative pathway, it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb. The active subunit Bb is a serine protease that associates with C3b to form the alternative pathway C3 convertase. Bb is involved in the proliferation of preactivated B lymphocytes, while Ba inhibits their proliferation. This gene localizes to the major histocompatibility complex (MHC class III) region on chromosome 6. This cluster includes several genes involved in regulation of the immune reaction. The polyadenylation site of this gene is 421 bp from the 5' end of the gene for complement component 2.[5]
Ambrus JL, Peters MG, Fauci AS, Brown EJ (March 1990). "The Ba fragment of complement factor B inhibits human B lymphocyte proliferation". Journal of Immunology. 144 (5): 1549–53. PMID 2307833.
Bradley DT, Zipfel PF, Hughes AE (June 2011). "Complement in age-related macular degeneration: a focus on function". Eye. 25 (6): 683–93. doi:10.1038/eye.2011.37. PMC3178140. PMID 21394116.
Campbell RD (January 1987). "The molecular genetics and polymorphism of C2 and factor B". British Medical Bulletin. 43 (1): 37–49. doi:10.1093/oxfordjournals.bmb.a072175. PMID 3315100.
Campbell RD, Bentley DR, Morley BJ (September 1984). "The factor B and C2 genes". Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences. 306 (1129): 367–78. Bibcode:1984RSPTB.306..367C. doi:10.1098/rstb.1984.0097. PMID 6149579.
Yu CY (1999). "Molecular genetics of the human MHC complement gene cluster". Experimental and Clinical Immunogenetics. 15 (4): 213–30. doi:10.1159/000019075. PMID 10072631. S2CID 25061446.
Rawal N, Pangburn MK (March 2001). "Structure/function of C5 convertases of complement". International Immunopharmacology. 1 (3): 415–22. doi:10.1016/S1567-5769(00)00039-4. PMID 11367526.
Arnason A, Larsen B, Marshall WH, Edwards JH, Macintosh P, Olaisen B, Teisberg P (August 1977). "Very close linkage between HLA-B and Bf inferred from allelic association". Nature. 268 (5620): 527–8. Bibcode:1977Natur.268..527A. doi:10.1038/268527a0. PMID 889587. S2CID 4262433.
Barnum SR, Ishii Y, Agrawal A, Volanakis JE (October 1992). "Production and interferon-gamma-mediated regulation of complement component C2 and factors B and D by the astroglioma cell line U105-MG". The Biochemical Journal. 287. 287 ( Pt 2) (2): 595–601. doi:10.1042/bj2870595. PMC1133207. PMID 1445220.
Yancey KB, Overholser O, Domloge-Hultsch N, Li LJ, Caughman SW, Bisalbutra P (March 1992). "Human keratinocytes and A-431 cells synthesize and secrete factor B, the major zymogen protease of the alternative complement pathway". The Journal of Investigative Dermatology. 98 (3): 379–83. doi:10.1111/1523-1747.ep12499812. PMID 1545147.
Niemann MA, Bhown AS, Miller EJ (March 1991). "The principal site of glycation of human complement factor B". The Biochemical Journal. 274. 274 ( Pt 2) (2): 473–80. doi:10.1042/bj2740473. PMC1150163. PMID 2006911.
Davrinche C, Abbal M, Clerc A (1991). "Molecular characterization of human complement factor B subtypes". Immunogenetics. 32 (5): 309–12. doi:10.1007/bf00211644. PMID 2249879. S2CID 13698345.
Farries TC, Lachmann PJ, Harrison RA (August 1988). "Analysis of the interaction between properdin and factor B, components of the alternative-pathway C3 convertase of complement". The Biochemical Journal. 253 (3): 667–75. doi:10.1042/bj2530667. PMC1149358. PMID 3140783.
Wu LC, Morley BJ, Campbell RD (January 1987). "Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements". Cell. 48 (2): 331–42. doi:10.1016/0092-8674(87)90436-3. PMID 3643061. S2CID 32752642.
Campbell RD, Porter RR (July 1983). "Molecular cloning and characterization of the gene coding for human complement protein factor B". Proceedings of the National Academy of Sciences of the United States of America. 80 (14): 4464–8. Bibcode:1983PNAS...80.4464C. doi:10.1073/pnas.80.14.4464. PMC384059. PMID 6308626.
Morley BJ, Campbell RD (January 1984). "Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen". The EMBO Journal. 3 (1): 153–7. doi:10.1002/j.1460-2075.1984.tb01776.x. PMC557312. PMID 6323161.
Christie DL, Gagnon J (January 1983). "Amino acid sequence of the Bb fragment from complement Factor B. Sequence of the major cyanogen bromide-cleavage peptide (CB-II) and completion of the sequence of the Bb fragment". The Biochemical Journal. 209 (1): 61–70. doi:10.1042/bj2090061. PMC1154056. PMID 6342610.
Mole JE, Anderson JK, Davison EA, Woods DE (March 1984). "Complete primary structure for the zymogen of human complement factor B". The Journal of Biological Chemistry. 259 (6): 3407–12. doi:10.1016/S0021-9258(17)43108-5. PMID 6546754.
Woods DE, Markham AF, Ricker AT, Goldberger G, Colten HR (September 1982). "Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product". Proceedings of the National Academy of Sciences of the United States of America. 79 (18): 5661–5. Bibcode:1982PNAS...79.5661W. doi:10.1073/pnas.79.18.5661. PMC346964. PMID 6957884.
Maruyama K, Sugano S (January 1994). "Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides". Gene. 138 (1–2): 171–4. doi:10.1016/0378-1119(94)90802-8. PMID 8125298.
Mejía JE, Jahn I, de la Salle H, Hauptmann G (January 1994). "Human factor B. Complete cDNA sequence of the BF*S allele". Human Immunology. 39 (1): 49–53. doi:10.1016/0198-8859(94)90100-7. PMID 8181962.
External linksEdit
GeneReviews/NCBI/NIH/UW entry on Atypical Hemolytic-Uremic Syndrome
OMIM entries on Atypical Hemolytic-Uremic Syndrome
The MEROPS online database for peptidases and their inhibitors: S01.196
complement, factor, protein, that, humans, encoded, gene, cfbavailable, structurespdbortholog, search, pdbe, rcsblist, codes3hs0, 1dle, 1q0p, 1rrk, 1rs0, 1rtk, 2ok5, 2win, 2xwb, 2xwj, 3hrzidentifiersaliasescfb, ai195813, ai255840, ahus4, armd14, cfab, cfbd, fb. Complement factor B is a protein that in humans is encoded by the CFB gene 5 CFBAvailable structuresPDBOrtholog search PDBe RCSBList of PDB id codes3HS0 1DLE 1Q0P 1RRK 1RS0 1RTK 2OK5 2WIN 2XWB 2XWJ 3HRZIdentifiersAliasesCFB Cfb AI195813 AI255840 Bf C2 Fb H2 Bf AHUS4 ARMD14 BFD CFAB CFBD FBI12 GBG PBF2 complement factor BExternal IDsOMIM 138470 MGI 105975 HomoloGene 1292 GeneCards CFBGene location Human Chr Chromosome 6 human 1 Band6p21 33Start31 945 650 bp 1 End31 952 084 bp 1 Gene location Mouse Chr Chromosome 17 mouse 2 Band17 B1 17 18 41 cMStart35 075 350 bp 2 End35 081 494 bp 2 RNA expression patternBgeeHumanMouse ortholog Top expressed inright lobe of livergallbladderappendixduodenumislet of Langerhansgastric mucosaright coronary arterykidneyupper lobe of left lungascending aortaTop expressed inproximal tubulekidneywhite adipose tissuehepatobiliary systemliverjejunumileumcolonduodenumspleenMore reference expression dataBioGPSMore reference expression dataGene ontologyMolecular functionpeptidase activity serine type peptidase activity serine type endopeptidase activity hydrolase activity complement binding protein bindingCellular componentextracellular region plasma membrane blood microparticle extracellular exosome extracellular spaceBiological processcomplement activation regulation of complement activation proteolysis immune system process complement activation alternative pathway innate immune responseSources Amigo QuickGOOrthologsSpeciesHumanMouseEntrez62914962EnsemblENSG00000243570ENSG00000239754ENSG00000243649ENSG00000241253ENSG00000242335ENSG00000241534ENSG00000204359ENSMUSG00000090231UniProtP00751P04186RefSeq mRNA NM 001710NM 001142706NM 008198RefSeq protein NP 001701NP 001136178NP 032224NP 001389804Location UCSC Chr 6 31 95 31 95 MbChr 17 35 08 35 08 MbPubMed search 3 4 WikidataView Edit HumanView Edit Mouse Contents 1 Function 2 References 3 Further reading 4 External linksFunction EditThis gene encodes complement factor B a component of the alternative pathway of complement activation Factor B circulates in the blood as a single chain polypeptide Upon activation of the alternative pathway it is cleaved by complement factor D yielding the noncatalytic chain Ba and the catalytic subunit Bb The active subunit Bb is a serine protease that associates with C3b to form the alternative pathway C3 convertase Bb is involved in the proliferation of preactivated B lymphocytes while Ba inhibits their proliferation This gene localizes to the major histocompatibility complex MHC class III region on chromosome 6 This cluster includes several genes involved in regulation of the immune reaction The polyadenylation site of this gene is 421 bp from the 5 end of the gene for complement component 2 5 nbsp Alternative pathway Some labels are in Polish Complement tests C4 C FB A C3 CH50 Conditions PSG C3 NeF AA HAE C4D TCPD SLE inflammationReferences Edit a b c ENSG00000239754 ENSG00000243649 ENSG00000241253 ENSG00000242335 ENSG00000241534 ENSG00000204359 GRCh38 Ensembl release 89 ENSG00000243570 ENSG00000239754 ENSG00000243649 ENSG00000241253 ENSG00000242335 ENSG00000241534 ENSG00000204359 Ensembl May 2017 a b c GRCm38 Ensembl release 89 ENSMUSG00000090231 Ensembl May 2017 Human PubMed Reference National Center for Biotechnology Information U S National Library of Medicine Mouse PubMed Reference National Center for Biotechnology Information U S National Library of Medicine a b Entrez Gene CFB complement factor B Further reading EditAmbrus JL Peters MG Fauci AS Brown EJ March 1990 The Ba fragment of complement factor B inhibits human B lymphocyte proliferation Journal of Immunology 144 5 1549 53 PMID 2307833 Bradley DT Zipfel PF Hughes AE June 2011 Complement in age related macular degeneration a focus on function Eye 25 6 683 93 doi 10 1038 eye 2011 37 PMC 3178140 PMID 21394116 Campbell RD January 1987 The molecular genetics and polymorphism of C2 and factor B British Medical Bulletin 43 1 37 49 doi 10 1093 oxfordjournals bmb a072175 PMID 3315100 Campbell RD Bentley DR Morley BJ September 1984 The factor B and C2 genes Philosophical Transactions of the Royal Society of London Series B Biological Sciences 306 1129 367 78 Bibcode 1984RSPTB 306 367C doi 10 1098 rstb 1984 0097 PMID 6149579 Yu CY 1999 Molecular genetics of the human MHC complement gene cluster Experimental and Clinical Immunogenetics 15 4 213 30 doi 10 1159 000019075 PMID 10072631 S2CID 25061446 Rawal N Pangburn MK March 2001 Structure function of C5 convertases of complement International Immunopharmacology 1 3 415 22 doi 10 1016 S1567 5769 00 00039 4 PMID 11367526 Arnason A Larsen B Marshall WH Edwards JH Macintosh P Olaisen B Teisberg P August 1977 Very close linkage between HLA B and Bf inferred from allelic association Nature 268 5620 527 8 Bibcode 1977Natur 268 527A doi 10 1038 268527a0 PMID 889587 S2CID 4262433 Barnum SR Ishii Y Agrawal A Volanakis JE October 1992 Production and interferon gamma mediated regulation of complement component C2 and factors B and D by the astroglioma cell line U105 MG The Biochemical Journal 287 287 Pt 2 2 595 601 doi 10 1042 bj2870595 PMC 1133207 PMID 1445220 Yancey KB Overholser O Domloge Hultsch N Li LJ Caughman SW Bisalbutra P March 1992 Human keratinocytes and A 431 cells synthesize and secrete factor B the major zymogen protease of the alternative complement pathway The Journal of Investigative Dermatology 98 3 379 83 doi 10 1111 1523 1747 ep12499812 PMID 1545147 Niemann MA Bhown AS Miller EJ March 1991 The principal site of glycation of human complement factor B The Biochemical Journal 274 274 Pt 2 2 473 80 doi 10 1042 bj2740473 PMC 1150163 PMID 2006911 Davrinche C Abbal M Clerc A 1991 Molecular characterization of human complement factor B subtypes Immunogenetics 32 5 309 12 doi 10 1007 bf00211644 PMID 2249879 S2CID 13698345 Farries TC Lachmann PJ Harrison RA August 1988 Analysis of the interaction between properdin and factor B components of the alternative pathway C3 convertase of complement The Biochemical Journal 253 3 667 75 doi 10 1042 bj2530667 PMC 1149358 PMID 3140783 Wu LC Morley BJ Campbell RD January 1987 Cell specific expression of the human complement protein factor B gene evidence for the role of two distinct 5 flanking elements Cell 48 2 331 42 doi 10 1016 0092 8674 87 90436 3 PMID 3643061 S2CID 32752642 Campbell RD Porter RR July 1983 Molecular cloning and characterization of the gene coding for human complement protein factor B Proceedings of the National Academy of Sciences of the United States of America 80 14 4464 8 Bibcode 1983PNAS 80 4464C doi 10 1073 pnas 80 14 4464 PMC 384059 PMID 6308626 Morley BJ Campbell RD January 1984 Internal homologies of the Ba fragment from human complement component Factor B a class III MHC antigen The EMBO Journal 3 1 153 7 doi 10 1002 j 1460 2075 1984 tb01776 x PMC 557312 PMID 6323161 Christie DL Gagnon J January 1983 Amino acid sequence of the Bb fragment from complement Factor B Sequence of the major cyanogen bromide cleavage peptide CB II and completion of the sequence of the Bb fragment The Biochemical Journal 209 1 61 70 doi 10 1042 bj2090061 PMC 1154056 PMID 6342610 Mole JE Anderson JK Davison EA Woods DE March 1984 Complete primary structure for the zymogen of human complement factor B The Journal of Biological Chemistry 259 6 3407 12 doi 10 1016 S0021 9258 17 43108 5 PMID 6546754 Woods DE Markham AF Ricker AT Goldberger G Colten HR September 1982 Isolation of cDNA clones for the human complement protein factor B a class III major histocompatibility complex gene product Proceedings of the National Academy of Sciences of the United States of America 79 18 5661 5 Bibcode 1982PNAS 79 5661W doi 10 1073 pnas 79 18 5661 PMC 346964 PMID 6957884 Maruyama K Sugano S January 1994 Oligo capping a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides Gene 138 1 2 171 4 doi 10 1016 0378 1119 94 90802 8 PMID 8125298 Mejia JE Jahn I de la Salle H Hauptmann G January 1994 Human factor B Complete cDNA sequence of the BF S allele Human Immunology 39 1 49 53 doi 10 1016 0198 8859 94 90100 7 PMID 8181962 External links EditGeneReviews NCBI NIH UW entry on Atypical Hemolytic Uremic Syndrome OMIM entries on Atypical Hemolytic Uremic Syndrome The MEROPS online database for peptidases and their inhibitors S01 196 Complement Factor B at the U S National Library of Medicine Medical Subject Headings MeSH Overview of all the structural information available in the PDB for UniProt P00751 Complement factor B at the PDBe KB nbsp This hydrolase article is a stub You can help Wikipedia by expanding it vte Retrieved from https en wikipedia org w index php title Complement factor B amp oldid 1170993039, wikipedia, wiki, book, books, library,
