BF

(B-Factor, properdin)

 

  • Alias                                 (According to NCBI)

 

  • CFB 
  • GBG 
  • PBF2
  • B-factor, properdin 
  • C3 proaccelerator
  • C3 proactivator 
  • C3/C5 convertase 
  • glycine-rich beta-glycoprotein 
  • Complement factor B precursor (EC 3.4.21.47) (C3/C5 convertase) (Properdin factor B) (Glycine-rich
    beta glycoprotein) (GBG) (PBF2).
  • 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 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.
  • Location: 6p21.3
  • Orientation: plus strand
  • Size:6079 bp 
  • 18 exons
  • DNA sequence (Human): NC_000006.

  • CGH (6p21.3):  Losses (%) -1.1   Gain (%)  9.5   Amplifications  0.0

  • Mutations and SNPs (According to HGMD and SNP)

 

  • HGMD :
  • SNPs: BF
  • m-RNA                       (According to NCBI and CGAP)

 

  • mRNA sequence (Human): NM_001710.

  • Size:   2565 bp  

  • cDNA libraries: BF

  • Size: 764 amino acids; 85532 Da
  • Catalytic activity: Cleavage of Arg-|-Ser bond in complement component C3 alpha-chain to yield C3a and C3b, and Arg-|-Xaa bond in complement component C5 alpha-chain to yield C5a and C5b.
  • Subcellular location: Secreted.

  • Protein domains

 

                                

  • Pathways and interactions (According to BioCarta, DIP)

                          Complement Pathway

 

  • Protein Interactions:

  • Clinical                            (According to OMIM, PubMed)