complement factor B

Target id: 2339

Nomenclature: complement factor B

Family: S1: Chymotrypsin

Annotation status:  image of a grey circle Awaiting annotation/under development. Please contact us if you can help with annotation.  » Email us

   GtoImmuPdb view: OFF :     complement factor B has curated GtoImmuPdb data

Gene and Protein Information
Species TM AA Chromosomal Location Gene Symbol Gene Name Reference
Human - 764 6p21.3 CFB complement factor B
Mouse - 763 17 B1 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
BRENDA
CATH/Gene3D
ChEMBL Target
Ensembl Gene
Entrez Gene
GenitoUrinary Development Molecular Anatomy Project
Human Protein Atlas
KEGG Enzyme
KEGG Gene
OMIM
Orphanet
RefSeq Nucleotide
RefSeq Protein
UniProtKB
Wikipedia
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 Affinity Units Reference
compound 51 [PMID: 19743866] Hs Inhibition 6.6 pIC50 2
pIC50 6.6 (IC50 2.5x10-7 M) [2]
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 ID:  2
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  complement activation, alternative pathway (GO:0006957) TAS
regulation of complement activation (GO:0030449) TAS
References: 
Immuno Process:  Immune regulation
Immuno Process ID:  6
Comment: 
GO Annotation:  Associated to GO processes
GO Processes:  complement activation (GO:0006956) TAS
complement activation, alternative pathway (GO:0006957) TAS
regulation of complement activation (GO:0030449) TAS
References: 
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: 
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

References

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1. Gold B, Merriam JE, Zernant J, Hancox LS, Taiber AJ, Gehrs K, Cramer K, Neel J, Bergeron J, Barile GR et al.. (2006) Variation in factor B (BF) and complement component 2 (C2) genes is associated with age-related macular degeneration. Nat. Genet.38 (4): 458-62. [PMID:16518403]

2. Ruiz-Gómez G, Lim J, Halili MA, Le GT, Madala PK, Abbenante G, Fairlie DP. (2009) Structure-activity relationships for substrate-based inhibitors of human complement factor B. J. Med. Chem.52 (19): 6042-52. [PMID:19743866]

How to cite this page

S1: Chymotrypsin: complement factor B. Last modified on 17/02/2017. Accessed on 22/10/2017. IUPHAR/BPS Guide to PHARMACOLOGY, http://www.guidetopharmacology.org/GRAC/ObjectDisplayForward?objectId=2339.