Complement factor B

Protein-coding gene in the species Homo sapiens
CFB
Available structures
PDBOrtholog search: PDBe RCSB
List of PDB id codes

3HS0, 1DLE, 1Q0P, 1RRK, 1RS0, 1RTK, 2OK5, 2WIN, 2XWB, 2XWJ, 3HRZ

Identifiers
AliasesCFB, Cfb, AI195813, AI255840, Bf, C2, Fb, H2-Bf, AHUS4, ARMD14, BFD, CFAB, CFBD, FBI12, GBG, PBF2, complement factor B
External IDsOMIM: 138470; MGI: 105975; HomoloGene: 1292; GeneCards: CFB; OMA:CFB - orthologs
Gene location (Human)
Chromosome 6 (human)
Chr.Chromosome 6 (human)[1]
Chromosome 6 (human)
Genomic location for CFB
Genomic location for CFB
Band6p21.33Start31,945,650 bp[1]
End31,952,084 bp[1]
Gene location (Mouse)
Chromosome 17 (mouse)
Chr.Chromosome 17 (mouse)[2]
Chromosome 17 (mouse)
Genomic location for CFB
Genomic location for CFB
Band17 B1|17 18.41 cMStart35,075,350 bp[2]
End35,081,494 bp[2]
RNA expression pattern
Bgee
HumanMouse (ortholog)
Top expressed in
  • right lobe of liver

  • gallbladder

  • appendix

  • duodenum

  • islet of Langerhans

  • olfactory zone of nasal mucosa

  • Descending thoracic aorta

  • gastric mucosa

  • right coronary artery

  • human kidney
Top expressed in
  • proximal tubule

  • right kidney

  • human kidney

  • white adipose tissue

  • hepatobiliary system

  • liver

  • jejunum

  • ileum

  • colon

  • duodenum
More reference expression data
BioGPS


More reference expression data
Gene ontology
Molecular function
  • peptidase activity
  • serine-type peptidase activity
  • serine-type endopeptidase activity
  • hydrolase activity
  • complement binding
  • protein binding
Cellular component
  • extracellular region
  • plasma membrane
  • blood microparticle
  • extracellular exosome
  • extracellular space
Biological process
  • complement activation
  • regulation of complement activation
  • proteolysis
  • immune system process
  • complement activation, alternative pathway
  • innate immune response
Sources:Amigo / QuickGO
Orthologs
SpeciesHumanMouse
Entrez

629

14962

Ensembl
ENSG00000243570
ENSG00000239754
ENSG00000243649
ENSG00000241253
ENSG00000242335

ENSG00000241534
ENSG00000204359

ENSMUSG00000090231

UniProt

P00751

P04186

RefSeq (mRNA)

NM_001710

NM_001142706
NM_008198

RefSeq (protein)

NP_001701

NP_001136178
NP_032224
NP_001389804

Location (UCSC)Chr 6: 31.95 – 31.95 MbChr 17: 35.08 – 35.08 Mb
PubMed search[3][4]
Wikidata
View/Edit HumanView/Edit Mouse

Complement factor B is a protein that in humans is encoded by the CFB gene.[5]

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.[5]

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. ^ a b c ENSG00000239754, ENSG00000243649, ENSG00000241253, ENSG00000242335, ENSG00000241534, ENSG00000204359 GRCh38: Ensembl release 89: ENSG00000243570, ENSG00000239754, ENSG00000243649, ENSG00000241253, ENSG00000242335, ENSG00000241534, ENSG00000204359 – Ensembl, May 2017
  2. ^ a b c GRCm38: Ensembl release 89: ENSMUSG00000090231 – Ensembl, May 2017
  3. ^ "Human PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  4. ^ "Mouse PubMed Reference:". National Center for Biotechnology Information, U.S. National Library of Medicine.
  5. ^ a b "Entrez Gene: CFB complement factor B".

Further reading

  • 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. doi:10.4049/jimmunol.144.5.1549. 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.
  • 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 links

  • 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+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.


  • v
  • t
  • e
  • 1dle: FACTOR B SERINE PROTEASE DOMAIN
    1dle: FACTOR B SERINE PROTEASE DOMAIN
  • 1q0p: A domain of Factor B
    1q0p: A domain of Factor B
  • 1rrk: Crystal Structure Analysis of the Bb segment of Factor B
    1rrk: Crystal Structure Analysis of the Bb segment of Factor B
  • 1rs0: Crystal Structure Analysis of the Bb segment of Factor B complexed with Di-isopropyl-phosphate (DIP)
    1rs0: Crystal Structure Analysis of the Bb segment of Factor B complexed with Di-isopropyl-phosphate (DIP)
  • 1rtk: Crystal Structure Analysis of the Bb segment of Factor B complexed with 4-guanidinobenzoic acid
    1rtk: Crystal Structure Analysis of the Bb segment of Factor B complexed with 4-guanidinobenzoic acid
  • 2ok5: Human Complement factor B
    2ok5: Human Complement factor B
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