CFB
Synonyms
C3 Proaccelerator, C3 Proactivator, EC 3.4.21.47, ARMD14, AHUS4, FBI12, H2-Bf, CFAB, CFBD, FB, Complement Factor B, EC 3.4.21, Properdin factor B, BF, Glycine-rich beta glycoprotein, CFB, B-Factor, Properdin, C3/C5 Convertase, PBF2, BFD, GBG, Glycine-Rich Beta-Glycoprotein, EC:3.4.21.47
Description
Complement factor B (CFB) 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.
KO Status
F1 (+/-)
Drug Information
Drugs in clinical trials: 2
Latest Research Phase: Phase 3 Clinical
Drug Name
Code
Phase
Company
Indications
Clinical Trials
Iptacopan
LNP-023
Phase 3 Clinical
Novartis Pharma Ag
Glomerulonephritis, IGA, Hemoglobinuria, Paroxysmal, Glomerulonephritis, Membranous, Glomerulonephritis
IONIS-FB-LRx
IONIS-GSK4-LRx, ISIS-696844, ISIS-GSK4Rx, IONIS-FB-LRx, ISIS-GSK4-LRx
Phase 2 Clinical
Ionis
Glomerulonephritis, IGA, Geographic Atrophy, Macular Degeneration
FC-2.15
FC-2.15
Discontinued
University Of Buenos Aires
Neoplasms
References
Title
Authors
Source
The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)
The MGC Project Team,
Genome Res. 14:2121-2127(2004)
Complete primary structure for the zymogen of human complement factor B
Mole J.E., Anderson J.K., Davison E.A., Woods D.E.,
J. Biol. Chem. 259:3407-3412(1984)
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
Christie D.L., Gagnon J.,
Biochem. J. 209:61-70(1983)
Molecular cloning and characterization of the gene coding for human complement protein factor B
Campbell R.D., Porter R.R.,
Proc. Natl. Acad. Sci. U.S.A. 80:4464-4468(1983)
Isolation of cDNA clones for the human complement protein factor B, a class III major histocompatibility complex gene product
Woods D.E., Markham A.F., Ricker A.T., Goldberger G., Colten H.R.,
Proc. Natl. Acad. Sci. U.S.A. 79:5661-5665(1982)
Internal homologies of the Ba fragment from human complement component Factor B, a class III MHC antigen
Morley B.J., Campbell R.D.,
EMBO J. 3:153-157(1984)
Cell-specific expression of the human complement protein factor B gene: evidence for the role of two distinct 5'-flanking elements
Wu L.C., Morley B.J., Campbell R.D.,
Cell 48:331-342(1987)
The principal site of glycation of human complement factor B
Niemann M.A., Bhown A.S., Miller E.J.,
Biochem. J. 274:473-480(1991)
Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry
Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E., Moore R.J., Smith R.D.,
J. Proteome Res. 4:2070-2080(2005)
Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry
Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.,
J. Proteome Res. 8:651-661(2009)