Gene and Protein Information
Species	TM	AA	Chromosomal Location	Gene Symbol	Gene Name	Reference
Human	-	764	6p21.33	CFB	complement factor B
Mouse	-	761	17 18.41 cM	Cfb	complement factor B
Rat	-	763	20p12	Cfb	complement factor B

Previous and Unofficial Names

alternative-complement pathway C3/C5 convertase | B-factor, properdin | BFD | C2 | Factor B | H2-Bf | histocompatibility 2, complement component factor B | properdin factor B

Database Links
Specialist databases
MEROPS	S01.196 (Hs)
Other databases
Alphafold	P00751 (Hs), P04186 (Mm)
BRENDA	3.4.21.47
CATH/Gene3D	3.40.50.410
ChEMBL Target	CHEMBL5731 (Hs), CHEMBL4630808 (Mm)
Ensembl Gene	ENSG00000243649 (Hs), ENSMUSG00000090231 (Mm), ENSRNOG00000051235 (Rn)
Entrez Gene	629 (Hs), 14962 (Mm), 294257 (Rn)
Human Protein Atlas	ENSG00000243649 (Hs)
KEGG Enzyme	3.4.21.47
KEGG Gene	hsa:629 (Hs), mmu:14962 (Mm), rno:294257 (Rn)
OMIM	138470 (Hs)
Orphanet	ORPHA158379 (Hs)
Pharos	P00751 (Hs)
RefSeq Nucleotide	NM_001710 (Hs), NM_212466 (Rn)
RefSeq Protein	NP_001701 (Hs), NP_997631 (Rn)
UniProtKB	P00751 (Hs), P04186 (Mm)
Wikipedia	CFB (Hs)

Enzyme Reaction
EC Number: 3.4.21.47

Download all structure-activity data for this target as a CSV file

Inhibitors
Key to terms and symbols	View all chemical structures	Click column headers to sort
Ligand Sp. Action Value Parameter Reference iptacopan Hs Inhibition 7.9 pIC50 2 ⤷ pIC50 7.9 (IC50 1.2x10-8 M) [2] Description: Determined in a FRET competition binding assay with factor B and a Cy5-labeled small-molecule inhibitor as probe. compound 51 [PMID: 19743866] Hs Inhibition 6.6 pIC50 3 ⤷ pIC50 6.6 (IC50 2.5x10-7 M) [3]

Immunopharmacology Comments

A protease component of the alternative complement pathway. Cleaved by complement factor D into a non-catalytic and catalytic fragment (Ba and Bb respectively).

Immuno Process Associations

Immuno Process:  Inflammation

Immuno Process:  Immune regulation

Clinically-Relevant Mutations and Pathophysiology
Disease:  Complement factor B deficiency; CFBD OMIM: 615561
Disease:  Hemolytic uremic syndrome, atypical, susceptibility to, 4 Synonyms: Atypical hemolytic-uremic syndrome with B factor anomaly [Orphanet: ORPHA93578] Hemolytic-uremic syndrome [Disease Ontology: DOID:12554] Disease Ontology: DOID:12554 OMIM: 612924 Orphanet: ORPHA93578
Disease:  Macular degeneration, age-related, 14; ARMD14 Synonyms: Age-related macular degeneration [Orphanet: ORPHA279] [Disease Ontology: DOID:10871] Disease Ontology: DOID:10871 OMIM: 615489 Orphanet: ORPHA279 Role:  Reduced risk of age-related macular degeneration seen in individuals with the mutations outlined below References:  1 Click column headers to sort Type Species Amino acid change Nucleotide change Description Reference Missense Human L9H 26T>A This variant is associated with reduced risk of age-related macular degeneration when inherited with the E318D variant of the C2 gene as part of haplotype 10. 1