Complement factor B

Details

Name
Complement factor B
Synonyms
  • 3.4.21.47
  • BF
  • BFD
  • C3/C5 convertase
  • GBG
  • Glycine-rich beta glycoprotein
  • PBF2
  • Properdin factor B
Gene Name
CFB
Organism
Humans
Amino acid sequence
>lcl|BSEQ0003373|Complement factor B
MGSNLSPQLCLMPFILGLLSGGVTTTPWSLARPQGSCSLEGVEIKGGSFRLLQEGQALEY
VCPSGFYPYPVQTRTCRSTGSWSTLKTQDQKTVRKAECRAIHCPRPHDFENGEYWPRSPY
YNVSDEISFHCYDGYTLRGSANRTCQVNGRWSGQTAICDNGAGYCSNPGIPIGTRKVGSQ
YRLEDSVTYHCSRGLTLRGSQRRTCQEGGSWSGTEPSCQDSFMYDTPQEVAEAFLSSLTE
TIEGVDAEDGHGPGEQQKRKIVLDPSGSMNIYLVLDGSDSIGASNFTGAKKCLVNLIEKV
ASYGVKPRYGLVTYATYPKIWVKVSEADSSNADWVTKQLNEINYEDHKLKSGTNTKKALQ
AVYSMMSWPDDVPPEGWNRTRHVIILMTDGLHNMGGDPITVIDEIRDLLYIGKDRKNPRE
DYLDVYVFGVGPLVNQVNINALASKKDNEQHVFKVKDMENLEDVFYQMIDESQSLSLCGM
VWEHRKGTDYHKQPWQAKISVIRPSKGHESCMGAVVSEYFVLTAAHCFTVDDKEHSIKVS
VGGEKRDLEIEVVLFHPNYNINGKKEAGIPEFYDYDVALIKLKNKLKYGQTIRPICLPCT
EGTTRALRLPPTTTCQQQKEELLPAQDIKALFVSEEEKKLTRKEVYIKNGDKKGSCERDA
QYAPGYDKVKDISEVVTPRFLCTGGVSPYADPNTCRGDSGGPLIVHKRSRFIQVGVISWG
VVDVCKNQKRQKQVPAHARDFHINLFQVLPWLKEKLQDEDLGFL
Number of residues
764
Molecular Weight
85532.325
Theoretical pI
7.07
GO Classification
Functions
complement binding / serine-type endopeptidase activity
Processes
complement activation / complement activation, alternative pathway / innate immune response / regulation of complement activation
Components
blood microparticle / extracellular exosome / extracellular region / extracellular space / plasma membrane
General Function
Serine-type endopeptidase activity
Specific Function
Factor B which is part of the alternate pathway of the complement system is cleaved by factor D into 2 fragments: Ba and Bb. Bb, a serine protease, then combines with complement factor 3b to generate the C3 or C5 convertase. It has also been implicated in proliferation and differentiation of preactivated B-lymphocytes, rapid spreading of peripheral blood monocytes, stimulation of lymphocyte blastogenesis and lysis of erythrocytes. Ba inhibits the proliferation of preactivated B-lymphocytes.
Pfam Domain Function
Transmembrane Regions
Not Available
Cellular Location
Secreted
Gene sequence
>lcl|BSEQ0021670|Complement factor B (CFB)
ATGGGGAGCAATCTCAGCCCCCAACTCTGCCTGATGCCCTTTATCTTGGGCCTCTTGTCT
GGAGGTGTGACCACCACTCCATGGTCTTTGGCCCGGCCCCAGGGATCCTGCTCTCTGGAG
GGGGTAGAGATCAAAGGCGGCTCCTTCCGACTTCTCCAAGAGGGCCAGGCACTGGAGTAC
GTGTGTCCTTCTGGCTTCTACCCGTACCCTGTGCAGACACGTACCTGCAGATCTACGGGG
TCCTGGAGCACCCTGAAGACTCAAGACCAAAAGACTGTCAGGAAGGCAGAGTGCAGAGCA
ATCCACTGTCCAAGACCACACGACTTCGAGAACGGGGAATACTGGCCCCGGTCTCCCTAC
TACAATGTGAGTGATGAGATCTCTTTCCACTGCTATGACGGTTACACTCTCCGGGGCTCT
GCCAATCGCACCTGCCAAGTGAATGGCCGATGGAGTGGGCAGACAGCGATCTGTGACAAC
GGAGCGGGGTACTGCTCCAACCCGGGCATCCCCATTGGCACAAGGAAGGTGGGCAGCCAG
TACCGCCTTGAAGACAGCGTCACCTACCACTGCAGCCGGGGGCTTACCCTGCGTGGCTCC
CAGCGGCGAACGTGTCAGGAAGGTGGCTCTTGGAGCGGGACGGAGCCTTCCTGCCAAGAC
TCCTTCATGTACGACACCCCTCAAGAGGTGGCCGAAGCTTTCCTGTCTTCCCTGACAGAG
ACCATAGAAGGAGTCGATGCTGAGGATGGGCACGGCCCAGGGGAACAACAGAAGCGGAAG
ATCGTCCTGGACCCTTCAGGCTCCATGAACATCTACCTGGTGCTAGATGGATCAGACAGC
ATTGGGGCCAGCAACTTCACAGGAGCCAAAAAGTGTCTAGTCAACTTAATTGAGAAGGTG
GCAAGTTATGGTGTGAAGCCAAGATATGGTCTAGTGACATATGCCACATACCCCAAAATT
TGGGTCAAAGTGTCTGAAGCAGACAGCAGTAATGCAGACTGGGTCACGAAGCAGCTCAAT
GAAATCAATTATGAAGACCACAAGTTGAAGTCAGGGACTAACACCAAGAAGGCCCTCCAG
GCAGTGTACAGCATGATGAGCTGGCCAGATGACGTCCCTCCTGAAGGCTGGAACCGCACC
CGCCATGTCATCATCCTCATGACTGATGGATTGCACAACATGGGCGGGGACCCAATTACT
GTCATTGATGAGATCCGGGACTTGCTATACATTGGCAAGGATCGCAAAAACCCAAGGGAG
GATTATCTGGATGTCTATGTGTTTGGGGTCGGGCCTTTGGTGAACCAAGTGAACATCAAT
GCTTTGGCTTCCAAGAAAGACAATGAGCAACATGTGTTCAAAGTCAAGGATATGGAAAAC
CTGGAAGATGTTTTCTACCAAATGATCGATGAAAGCCAGTCTCTGAGTCTCTGTGGCATG
GTTTGGGAACACAGGAAGGGTACCGATTACCACAAGCAACCATGGCAGGCCAAGATCTCA
GTCATTCGCCCTTCAAAGGGACACGAGAGCTGTATGGGGGCTGTGGTGTCTGAGTACTTT
GTGCTGACAGCAGCACATTGTTTCACTGTGGATGACAAGGAACACTCAATCAAGGTCAGC
GTAGGAGGGGAGAAGCGGGACCTGGAGATAGAAGTAGTCCTATTTCACCCCAACTACAAC
ATTAATGGGAAAAAAGAAGCAGGAATTCCTGAATTTTATGACTATGACGTTGCCCTGATC
AAGCTCAAGAATAAGCTGAAATATGGCCAGACTATCAGGCCCATTTGTCTCCCCTGCACC
GAGGGAACAACTCGAGCTTTGAGGCTTCCTCCAACTACCACTTGCCAGCAACAAAAGGAA
GAGCTGCTCCCTGCACAGGATATCAAAGCTCTGTTTGTGTCTGAGGAGGAGAAAAAGCTG
ACTCGGAAGGAGGTCTACATCAAGAATGGGGATAAGAAAGGCAGCTGTGAGAGAGATGCT
CAATATGCCCCAGGCTATGACAAAGTCAAGGACATCTCAGAGGTGGTCACCCCTCGGTTC
CTTTGTACTGGAGGAGTGAGTCCCTATGCTGACCCCAATACTTGCAGAGGTGATTCTGGC
GGCCCCTTGATAGTTCACAAGAGAAGTCGTTTCATTCAAGTTGGTGTAATCAGCTGGGGA
GTAGTGGATGTCTGCAAAAACCAGAAGCGGCAAAAGCAGGTACCTGCTCACGCCCGAGAC
TTTCACATCAACCTCTTTCAAGTGCTGCCCTGGCTGAAGGAGAAACTCCAAGATGAGGAT
TTGGGTTTTCTATAA
Chromosome Location
6
Locus
6p21.3
External Identifiers
ResourceLink
UniProtKB IDP00751
UniProtKB Entry NameCFAB_HUMAN
GenBank Protein ID297569
GenBank Gene IDX72875
GenAtlas IDCFB
HGNC IDHGNC:1037
General References
  1. Davrinche C, Abbal M, Clerc A: Molecular characterization of human complement factor B subtypes. Immunogenetics. 1990;32(5):309-12. [PubMed:2249879]
  2. Mejia JE, Jahn I, de la Salle H, Hauptmann G: Human factor B. Complete cDNA sequence of the BF*S allele. Hum Immunol. 1994 Jan;39(1):49-53. [PubMed:8181962]
  3. Schwaeble W, Luttig B, Sokolowski T, Estaller C, Weiss EH, Meyer zum Buschenfelde KH, Whaley K, Dippold W: Human complement factor B: functional properties of a recombinant zymogen of the alternative activation pathway convertase. Immunobiology. 1993 Jul;188(3):221-32. [PubMed:8225386]
  4. Horiuchi T, Kim S, Matsumoto M, Watanabe I, Fujita S, Volanakis JE: Human complement factor B: cDNA cloning, nucleotide sequencing, phenotypic conversion by site-directed mutagenesis and expression. Mol Immunol. 1993 Dec;30(17):1587-92. [PubMed:8247029]
  5. Xie T, Rowen L, Aguado B, Ahearn ME, Madan A, Qin S, Campbell RD, Hood L: Analysis of the gene-dense major histocompatibility complex class III region and its comparison to mouse. Genome Res. 2003 Dec;13(12):2621-36. [PubMed:14656967]
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  8. Mole JE, Anderson JK, Davison EA, Woods DE: Complete primary structure for the zymogen of human complement factor B. J Biol Chem. 1984 Mar 25;259(6):3407-12. [PubMed:6546754]
  9. Christie DL, Gagnon J: 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. Biochem J. 1983 Jan 1;209(1):61-70. [PubMed:6342610]
  10. Campbell RD, Porter RR: Molecular cloning and characterization of the gene coding for human complement protein factor B. Proc Natl Acad Sci U S A. 1983 Jul;80(14):4464-8. [PubMed:6308626]
  11. Woods DE, Markham AF, Ricker AT, Goldberger G, Colten HR: Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product. Proc Natl Acad Sci U S A. 1982 Sep;79(18):5661-5. [PubMed:6957884]
  12. Morley BJ, Campbell RD: Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen. EMBO J. 1984 Jan;3(1):153-7. [PubMed:6323161]
  13. Wu LC, Morley BJ, Campbell RD: Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements. Cell. 1987 Jan 30;48(2):331-42. [PubMed:3643061]
  14. Niemann MA, Bhown AS, Miller EJ: The principal site of glycation of human complement factor B. Biochem J. 1991 Mar 1;274 ( Pt 2):473-80. [PubMed:2006911]
  15. Liu T, Qian WJ, Gritsenko MA, Camp DG 2nd, Monroe ME, Moore RJ, Smith RD: Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry. J Proteome Res. 2005 Nov-Dec;4(6):2070-80. [PubMed:16335952]
  16. Chen R, Jiang X, Sun D, Han G, Wang F, Ye M, Wang L, Zou H: Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry. J Proteome Res. 2009 Feb;8(2):651-61. doi: 10.1021/pr8008012. [PubMed:19159218]
  17. Slade C, Bosco J, Unglik G, Bleasel K, Nagel M, Winship I: Deficiency in complement factor B. N Engl J Med. 2013 Oct 24;369(17):1667-9. doi: 10.1056/NEJMc1306326. [PubMed:24152280]
  18. Jing H, Xu Y, Carson M, Moore D, Macon KJ, Volanakis JE, Narayana SV: New structural motifs on the chymotrypsin fold and their potential roles in complement factor B. EMBO J. 2000 Jan 17;19(2):164-73. [PubMed:10637221]
  19. Milder FJ, Gomes L, Schouten A, Janssen BJ, Huizinga EG, Romijn RA, Hemrika W, Roos A, Daha MR, Gros P: Factor B structure provides insights into activation of the central protease of the complement system. Nat Struct Mol Biol. 2007 Mar;14(3):224-8. Epub 2007 Feb 25. [PubMed:17310251]
  20. Janssen BJ, Gomes L, Koning RI, Svergun DI, Koster AJ, Fritzinger DC, Vogel CW, Gros P: Insights into complement convertase formation based on the structure of the factor B-cobra venom factor complex. EMBO J. 2009 Aug 19;28(16):2469-78. doi: 10.1038/emboj.2009.184. Epub 2009 Jul 2. [PubMed:19574954]
  21. Gold B, Merriam JE, Zernant J, Hancox LS, Taiber AJ, Gehrs K, Cramer K, Neel J, Bergeron J, Barile GR, Smith RT, Hageman GS, Dean M, Allikmets R: Variation in factor B (BF) and complement component 2 (C2) genes is associated with age-related macular degeneration. Nat Genet. 2006 Apr;38(4):458-62. Epub 2006 Mar 5. [PubMed:16518403]
  22. Goicoechea de Jorge E, Harris CL, Esparza-Gordillo J, Carreras L, Arranz EA, Garrido CA, Lopez-Trascasa M, Sanchez-Corral P, Morgan BP, Rodriguez de Cordoba S: Gain-of-function mutations in complement factor B are associated with atypical hemolytic uremic syndrome. Proc Natl Acad Sci U S A. 2007 Jan 2;104(1):240-5. Epub 2006 Dec 20. [PubMed:17182750]
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Drug Relations

Drug Relations
DrugBank IDNameDrug groupPharmacological action?ActionsDetails
DB024594-Guanidinobenzoic AcidexperimentalunknownDetails
DB04491Diisopropylphosphono GroupexperimentalunknownDetails
DB06503MLN2222investigationalunknownDetails
DB01593Zincapproved, investigationalunknownDetails
DB14487Zinc acetateapproved, investigationalunknownDetails
DB14533Zinc chlorideapproved, investigationalunknownDetails