Complement factor B: Difference between revisions

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{{Infobox_gene}}


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'''Complement factor B''' is a [[protein]] that in humans is encoded by the ''CFB'' [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: CFB complement factor B| url = https://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=629| accessdate = }}</ref>
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| update_protein_box = yes
| update_summary = yes
| update_citations = yes
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<!-- The GNF_Protein_box is automatically maintained by Protein Box Bot.  See Template:PBB_Controls to Stop updates. -->
== Function ==
{{GNF_Protein_box
| image = PBB_Protein_CFB_image.jpg
| image_source = [[Protein_Data_Bank|PDB]] rendering based on 1dle.
| PDB = {{PDB2|1dle}}, {{PDB2|1q0p}}, {{PDB2|1rrk}}, {{PDB2|1rs0}}, {{PDB2|1rtk}}, {{PDB2|2ok5}}
| Name = Complement factor B
| HGNCid = 1037
| Symbol = CFB
| AltSymbols =; BF; BFD; CFAB; GBG; H2-Bf; PBF2
| OMIM = 138470
| ECnumber = 
| Homologene = 1292
| MGIid = 105975
| GeneAtlas_image1 = PBB_GE_CFB_202357_s_at_tn.png
| GeneAtlas_image2 = PBB_GE_CFB_211920_at_tn.png
| Function = {{GNF_GO|id=GO:0001848 |text = complement binding}} {{GNF_GO|id=GO:0003812 |text = alternative-complement-pathway C3/C5 convertase activity}} {{GNF_GO|id=GO:0004252 |text = serine-type endopeptidase activity}} {{GNF_GO|id=GO:0008233 |text = peptidase activity}}
| Component = {{GNF_GO|id=GO:0005576 |text = extracellular region}}
| Process = {{GNF_GO|id=GO:0006508 |text = proteolysis}} {{GNF_GO|id=GO:0006956 |text = complement activation}} {{GNF_GO|id=GO:0006957 |text = complement activation, alternative pathway}}
| Orthologs = {{GNF_Ortholog_box
    | Hs_EntrezGene = 629
    | Hs_Ensembl = ENSG00000166285
    | Hs_RefseqProtein = NP_001701
    | Hs_RefseqmRNA = NM_001710
    | Hs_GenLoc_db = 
    | Hs_GenLoc_chr = c6_COX
    | Hs_GenLoc_start = 32048579
    | Hs_GenLoc_end = 32054666
    | Hs_Uniprot = P00751
    | Mm_EntrezGene = 14962
    | Mm_Ensembl = ENSMUSG00000024371
    | Mm_RefseqmRNA = NM_008198
    | Mm_RefseqProtein = NP_032224
    | Mm_GenLoc_db = 
    | Mm_GenLoc_chr = 17
    | Mm_GenLoc_start = 34464437
    | Mm_GenLoc_end = 34470273
    | Mm_Uniprot = Q3U925
  }}
}}
[[Image:Droga alternatywna.png|thumb|right|500px|Alternative pathway. (Some labels are in Polish.)]]


'''Complement factor B''', also known as '''CFB''', is a human [[gene]].<ref name="entrez">{{cite web | title = Entrez Gene: CFB complement factor B| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=629| accessdate = }}</ref>
This gene encodes complement factor B, a component of the [[alternative complement pathway|alternative pathway]] of [[complement system|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 (human)|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.<ref name="entrez" />


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{{clear|left}}
{{PBB_Summary
[[Image:Droga alternatywna.png|thumb|left|500px|Alternative pathway. (Some labels are in Polish.)]]
| section_title =
{{clear|left}}
| summary_text = This gene encodes complement factor B, a component of the [[alternative complement pathway|alternative pathway]] of [[complement system|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 which 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.<ref name="entrez">{{cite web | title = Entrez Gene: CFB complement factor B| url = http://www.ncbi.nlm.nih.gov/sites/entrez?Db=gene&Cmd=ShowDetailView&TermToSearch=629| accessdate = }}</ref>
{{Complement test comparisons|align=left}}
}}
{{clear}}


==References==
== References ==
{{reflist}}
{{reflist}}


==Further reading==
== Further reading ==
{{refbegin | 2}}
{{refbegin | 2}}
{{PBB_Further_reading
* {{cite journal | vauthors = Ambrus JL, Peters MG, Fauci AS, Brown EJ | title = The Ba fragment of complement factor B inhibits human B lymphocyte proliferation | journal = Journal of Immunology | volume = 144 | issue = 5 | pages = 1549–53 | date = March 1990 | pmid = 2307833 | doi =  }}
| citations =  
* {{cite journal | vauthors = Bradley DT, Zipfel PF, Hughes AE | title = Complement in age-related macular degeneration: a focus on function | journal = Eye | volume = 25 | issue = 6 | pages = 683–93 | date = June 2011 | pmid = 21394116 | pmc = 3178140 | doi = 10.1038/eye.2011.37 }}
*{{cite journal | author=Campbell RD |title=The molecular genetics and polymorphism of C2 and factor B. |journal=Br. Med. Bull. |volume=43 |issue= 1 |pages= 37-49 |year= 1988 |pmid= 3315100 |doi=  }}
* {{cite journal | vauthors = Campbell RD | title = The molecular genetics and polymorphism of C2 and factor B | journal = British Medical Bulletin | volume = 43 | issue = 1 | pages = 37–49 | date = January 1987 | pmid = 3315100 | doi =  }}
*{{cite journal | author=Campbell RD, Bentley DR, Morley BJ |title=The factor B and C2 genes. |journal=Philos. Trans. R. Soc. Lond., B, Biol. Sci. |volume=306 |issue= 1129 |pages= 367-78 |year= 1984 |pmid= 6149579 |doi= }}
* {{cite journal | vauthors = Campbell RD, Bentley DR, Morley BJ | title = The factor B and C2 genes | journal = Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences | volume = 306 | issue = 1129 | pages = 367–78 | date = September 1984 | pmid = 6149579 | doi = 10.1098/rstb.1984.0097 }}
*{{cite journal | author=Yu CY |title=Molecular genetics of the human MHC complement gene cluster. |journal=Exp. Clin. Immunogenet. |volume=15 |issue= 4 |pages= 213-30 |year= 1999 |pmid= 10072631 |doi= }}
* {{cite journal | vauthors = Yu CY | title = Molecular genetics of the human MHC complement gene cluster | journal = Experimental and Clinical Immunogenetics | volume = 15 | issue = 4 | pages = 213–30 | year = 1999 | pmid = 10072631 | doi = 10.1159/000019075 }}
*{{cite journal | author=Rawal N, Pangburn MK |title=Structure/function of C5 convertases of complement. |journal=Int. Immunopharmacol. |volume=1 |issue= 3 |pages= 415-22 |year= 2001 |pmid= 11367526 |doi= }}
* {{cite journal | vauthors = Rawal N, Pangburn MK | title = Structure/function of C5 convertases of complement | journal = International Immunopharmacology | volume = 1 | issue = 3 | pages = 415–22 | date = March 2001 | pmid = 11367526 | doi = 10.1016/S1567-5769(00)00039-4 }}
*{{cite journal | author=Arnason A, Larsen B, Marshall WH, ''et al.'' |title=Very close linkage between HLA-B and Bf inferred from allelic association. |journal=Nature |volume=268 |issue= 5620 |pages= 527-8 |year= 1977 |pmid= 889587 |doi= }}
* {{cite journal | vauthors = Arnason A, Larsen B, Marshall WH, Edwards JH, Macintosh P, Olaisen B, Teisberg P | title = Very close linkage between HLA-B and Bf inferred from allelic association | journal = Nature | volume = 268 | issue = 5620 | pages = 527–8 | date = August 1977 | pmid = 889587 | doi = 10.1038/268527a0 }}
*{{cite journal | author=Barnum SR, Ishii Y, Agrawal A, Volanakis JE |title=Production and interferon-gamma-mediated regulation of complement component C2 and factors B and D by the astroglioma cell line U105-MG. |journal=Biochem. J. |volume=287 ( Pt 2) |issue=  |pages= 595-601 |year= 1992 |pmid= 1445220 |doi=  }}
* {{cite journal | vauthors = Barnum SR, Ishii Y, Agrawal A, Volanakis JE | title = Production and interferon-gamma-mediated regulation of complement component C2 and factors B and D by the astroglioma cell line U105-MG | journal = The Biochemical Journal | volume = 287 ( Pt 2) | issue =  | pages = 595–601 | date = October 1992 | pmid = 1445220 | pmc = 1133207 | doi =  | series = 287 }}
*{{cite journal | author=Yancey KB, Overholser O, Domloge-Hultsch N, ''et al.'' |title=Human keratinocytes and A-431 cells synthesize and secrete factor B, the major zymogen protease of the alternative complement pathway. |journal=J. Invest. Dermatol. |volume=98 |issue= 3 |pages= 379-83 |year= 1992 |pmid= 1545147 |doi= }}
* {{cite journal | vauthors = Yancey KB, Overholser O, Domloge-Hultsch N, Li LJ, Caughman SW, Bisalbutra P | title = Human keratinocytes and A-431 cells synthesize and secrete factor B, the major zymogen protease of the alternative complement pathway | journal = The Journal of Investigative Dermatology | volume = 98 | issue = 3 | pages = 379–83 | date = March 1992 | pmid = 1545147 | doi = 10.1111/1523-1747.ep12499812 }}
*{{cite journal | author=Niemann MA, Bhown AS, Miller EJ |title=The principal site of glycation of human complement factor B. |journal=Biochem. J. |volume=274 ( Pt 2) |issue=  |pages= 473-80 |year= 1991 |pmid= 2006911 |doi=  }}
* {{cite journal | vauthors = Niemann MA, Bhown AS, Miller EJ | title = The principal site of glycation of human complement factor B | journal = The Biochemical Journal | volume = 274 ( Pt 2) | issue =  | pages = 473–80 | date = March 1991 | pmid = 2006911 | pmc = 1150163 | doi =  | series = 274 }}
*{{cite journal | author=Davrinche C, Abbal M, Clerc A |title=Molecular characterization of human complement factor B subtypes. |journal=Immunogenetics |volume=32 |issue= 5 |pages= 309-12 |year= 1991 |pmid= 2249879 |doi= }}
* {{cite journal | vauthors = Davrinche C, Abbal M, Clerc A | title = Molecular characterization of human complement factor B subtypes | journal = Immunogenetics | volume = 32 | issue = 5 | pages = 309–12 | year = 1991 | pmid = 2249879 | doi = 10.1007/bf00211644 }}
*{{cite journal | author=Farries TC, Lachmann PJ, Harrison RA |title=Analysis of the interaction between properdin and factor B, components of the alternative-pathway C3 convertase of complement. |journal=Biochem. J. |volume=253 |issue= 3 |pages= 667-75 |year= 1988 |pmid= 3140783 |doi=  }}
* {{cite journal | vauthors = Farries TC, Lachmann PJ, Harrison RA | title = Analysis of the interaction between properdin and factor B, components of the alternative-pathway C3 convertase of complement | journal = The Biochemical Journal | volume = 253 | issue = 3 | pages = 667–75 | date = August 1988 | pmid = 3140783 | pmc = 1149358 | doi =  }}
*{{cite journal | author=Wu LC, Morley BJ, Campbell RD |title=Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements. |journal=Cell |volume=48 |issue= 2 |pages= 331-42 |year= 1987 |pmid= 3643061 |doi= }}
* {{cite journal | vauthors = Wu LC, Morley BJ, Campbell RD | title = Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements | journal = Cell | volume = 48 | issue = 2 | pages = 331–42 | date = January 1987 | pmid = 3643061 | doi = 10.1016/0092-8674(87)90436-3 }}
*{{cite journal | author=Campbell RD, Porter RR |title=Molecular cloning and characterization of the gene coding for human complement protein factor B. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=80 |issue= 14 |pages= 4464-8 |year= 1983 |pmid= 6308626 |doi= }}
* {{cite journal | vauthors = Campbell RD, Porter RR | title = Molecular cloning and characterization of the gene coding for human complement protein factor B | journal = Proceedings of the National Academy of Sciences of the United States of America | volume = 80 | issue = 14 | pages = 4464–8 | date = July 1983 | pmid = 6308626 | pmc = 384059 | doi = 10.1073/pnas.80.14.4464 }}
*{{cite journal | author=Morley BJ, Campbell RD |title=Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen. |journal=EMBO J. |volume=3 |issue= 1 |pages= 153-7 |year= 1984 |pmid= 6323161 |doi=  }}
* {{cite journal | vauthors = Morley BJ, Campbell RD | title = Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen | journal = The EMBO Journal | volume = 3 | issue = 1 | pages = 153–7 | date = January 1984 | pmid = 6323161 | pmc = 557312 | doi =  }}
*{{cite journal | author=Christie DL, Gagnon J |title=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. |journal=Biochem. J. |volume=209 |issue= 1 |pages= 61-70 |year= 1983 |pmid= 6342610 |doi=  }}
* {{cite journal | vauthors = Christie DL, Gagnon J | title = 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 | journal = The Biochemical Journal | volume = 209 | issue = 1 | pages = 61–70 | date = January 1983 | pmid = 6342610 | pmc = 1154056 | doi =  }}
*{{cite journal | author=Mole JE, Anderson JK, Davison EA, Woods DE |title=Complete primary structure for the zymogen of human complement factor B. |journal=J. Biol. Chem. |volume=259 |issue= 6 |pages= 3407-12 |year= 1984 |pmid= 6546754 |doi=  }}
* {{cite journal | vauthors = Mole JE, Anderson JK, Davison EA, Woods DE | title = Complete primary structure for the zymogen of human complement factor B | journal = The Journal of Biological Chemistry | volume = 259 | issue = 6 | pages = 3407–12 | date = March 1984 | pmid = 6546754 | doi =  }}
*{{cite journal | author=Woods DE, Markham AF, Ricker AT, ''et al.'' |title=Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product. |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=79 |issue= 18 |pages= 5661-5 |year= 1982 |pmid= 6957884 |doi= }}
* {{cite journal | vauthors = Woods DE, Markham AF, Ricker AT, Goldberger G, Colten HR | title = Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product | journal = Proceedings of the National Academy of Sciences of the United States of America | volume = 79 | issue = 18 | pages = 5661–5 | date = September 1982 | pmid = 6957884 | pmc = 346964 | doi = 10.1073/pnas.79.18.5661 }}
*{{cite journal | author=Maruyama K, Sugano S |title=Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides. |journal=Gene |volume=138 |issue= 1-2 |pages= 171-4 |year= 1994 |pmid= 8125298 |doi= }}
* {{cite journal | vauthors = Maruyama K, Sugano S | title = Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides | journal = Gene | volume = 138 | issue = 1-2 | pages = 171–4 | date = January 1994 | pmid = 8125298 | doi = 10.1016/0378-1119(94)90802-8 }}
*{{cite journal | author=Mejía JE, Jahn I, de la Salle H, Hauptmann G |title=Human factor B. Complete cDNA sequence of the BF*S allele. |journal=Hum. Immunol. |volume=39 |issue= 1 |pages= 49-53 |year= 1994 |pmid= 8181962 |doi= }}
* {{cite journal | vauthors = Mejía JE, Jahn I, de la Salle H, Hauptmann G | title = Human factor B. Complete cDNA sequence of the BF*S allele | journal = Human Immunology | volume = 39 | issue = 1 | pages = 49–53 | date = January 1994 | pmid = 8181962 | doi = 10.1016/0198-8859(94)90100-7 }}
}}
{{refend}}
{{refend}}


==External links==
== External links ==
* [https://www.ncbi.nlm.nih.gov/books/NBK1367/  GeneReviews/NCBI/NIH/UW entry on Atypical Hemolytic-Uremic Syndrome]
* [https://www.ncbi.nlm.nih.gov/omim/277400,120700,120920,134370,134371,138470,188040,217030,235400,605336,605337,612922,612923,612924,612925,612926,120700,120920,134370,134371,138470,188040,217030,235400,605336,605337,612922,612923,612924,612925,612926  OMIM entries on Atypical Hemolytic-Uremic Syndrome]
* The [[MEROPS]] online database for peptidases and their inhibitors: [http://merops.sanger.ac.uk/cgi-bin/merops.cgi?id=S01.196 S01.196]
* {{MeshName|Complement+Factor+B}}
* {{MeshName|Complement+Factor+B}}


{{PDB Gallery|geneid=629}}
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{{hydrolase-stub}}


[[pl:Czynnik B]]


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Latest revision as of 16:02, 30 August 2017

VALUE_ERROR (nil)
Identifiers
Aliases
External IDsGeneCards: [1]
Orthologs
SpeciesHumanMouse
Entrez

n/a

n/a

Ensembl

n/a

n/a

UniProt

n/a

n/a

RefSeq (mRNA)

n/a

n/a

RefSeq (protein)

n/a

n/a

Location (UCSC)n/an/a
PubMed searchn/an/a
Wikidata
View/Edit Human

Complement factor B is a protein that in humans is encoded by the CFB gene.[1]

Function

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.[1]

Alternative pathway. (Some labels are in Polish.)
Complement tests
C4 (C) FB (A) C3 CH50 Conditions
 · ↓ ↓ ↓ PSG, C3 NeF AA
↓  · ↓  · HAE, C4D
 ·  ·  · ↓ TCPD
↓  · /↓ ↓ ↓ SLE
↑ ↑ ↑ ↑ inflammation

References

  1. ↑ 1.0 1.1 "Entrez Gene: CFB complement factor B".

Further reading

External links

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