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)