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Protein

C4b-binding protein beta chain

Gene

C4BPB

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score: Annotation score: 5 out of 5-Experimental evidence at protein leveli

Functioni

Controls the classical pathway of complement activation. It binds as a cofactor to C3b/C4b inactivator (C3bINA), which then hydrolyzes the complement fragment C4b. It also accelerates the degradation of the C4bC2a complex (C3 convertase) by dissociating the complement fragment C2a. It also interacts with anticoagulant protein S and with serum amyloid P component. The beta chain binds protein S.

GO - Biological processi

  • blood coagulation Source: ProtInc
  • complement activation, classical pathway Source: UniProtKB-KW
  • innate immune response Source: UniProtKB-KW
  • negative regulation of complement activation, classical pathway Source: BHF-UCL
  • positive regulation of protein catabolic process Source: BHF-UCL
  • regulation of complement activation Source: Reactome
  • regulation of opsonization Source: BHF-UCL
Complete GO annotation...

Keywords - Biological processi

Complement pathway, Immunity, Innate immunity

Enzyme and pathway databases

BioCyciZFISH:ENSG00000123843-MONOMER.
ReactomeiR-HSA-977606. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
C4b-binding protein beta chain
Gene namesi
Name:C4BPB
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
Proteomesi
  • UP000005640 Componenti: Chromosome 1

Organism-specific databases

HGNCiHGNC:1328. C4BPB.

Subcellular locationi

GO - Cellular componenti

  • extracellular region Source: UniProtKB
  • extracellular space Source: BHF-UCL
  • other organism cell Source: ParkinsonsUK-UCL
  • plasma membrane Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Organism-specific databases

DisGeNETi725.
OpenTargetsiENSG00000123843.
PharmGKBiPA25908.

Polymorphism and mutation databases

BioMutaiC4BPB.
DMDMi115213.

PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Signal peptidei1 – 17Add BLAST17
ChainiPRO_000000589218 – 252C4b-binding protein beta chainAdd BLAST235

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Disulfide bondi23 ↔ 63PROSITE-ProRule annotation
Disulfide bondi49 ↔ 76PROSITE-ProRule annotation
Glycosylationi64N-linked (GlcNAc...)2 Publications1
Glycosylationi71N-linked (GlcNAc...)1 Publication1
Disulfide bondi81 ↔ 121PROSITE-ProRule annotation
Glycosylationi98N-linked (GlcNAc...)3 Publications1
Disulfide bondi107 ↔ 134PROSITE-ProRule annotation
Glycosylationi117N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi139 ↔ 179PROSITE-ProRule annotation
Glycosylationi154N-linked (GlcNAc...)Sequence analysis1
Disulfide bondi165 ↔ 191PROSITE-ProRule annotation
Disulfide bondi202Interchain (with alpha chain)PROSITE-ProRule annotation
Disulfide bondi216Interchain (with alpha chain)PROSITE-ProRule annotation

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP20851.
PaxDbiP20851.
PeptideAtlasiP20851.
PRIDEiP20851.

PTM databases

iPTMnetiP20851.
PhosphoSitePlusiP20851.

Expressioni

Gene expression databases

BgeeiENSG00000123843.
CleanExiHS_C4BPB.
ExpressionAtlasiP20851. baseline and differential.
GenevisibleiP20851. HS.

Organism-specific databases

HPAiHPA051620.

Interactioni

Subunit structurei

Disulfide-linked complex of alpha and beta chains of 3 possible sorts: a 570 kDa complex of 7 alpha chains and 1 beta chain, a 530 kDa homoheptamer of alpha chains or a 500 kDa complex of 6 alpha chains and 1 beta chain. The central body of the alpha chain homopolymer supports tentacles, each with the binding site for C4b at the end.

Protein-protein interaction databases

BioGridi107186. 15 interactors.
STRINGi9606.ENSP00000243611.

Structurei

3D structure databases

ProteinModelPortaliP20851.
SMRiP20851.
ModBaseiSearch...
MobiDBiSearch...

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Domaini21 – 78Sushi 1PROSITE-ProRule annotationAdd BLAST58
Domaini79 – 136Sushi 2PROSITE-ProRule annotationAdd BLAST58
Domaini137 – 193Sushi 3PROSITE-ProRule annotationAdd BLAST57

Sequence similaritiesi

Contains 3 Sushi (CCP/SCR) domains.PROSITE-ProRule annotation

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiKOG4297. Eukaryota.
ENOG410XPJ1. LUCA.
GeneTreeiENSGT00530000063826.
HOGENOMiHOG000032571.
HOVERGENiHBG004922.
InParanoidiP20851.
KOiK04003.
OMAiILKGSNW.
OrthoDBiEOG091G0JTL.
PhylomeDBiP20851.
TreeFamiTF342864.

Family and domain databases

CDDicd00033. CCP. 3 hits.
InterProiIPR000436. Sushi_SCR_CCP_dom.
[Graphical view]
PfamiPF00084. Sushi. 3 hits.
[Graphical view]
SMARTiSM00032. CCP. 3 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 3 hits.
PROSITEiPS50923. SUSHI. 3 hits.
[Graphical view]

Sequences (2)i

Sequence statusi: Complete.

Sequence processingi: The displayed sequence is further processed into a mature form.

This entry describes 2 isoformsi produced by alternative splicing. AlignAdd to basket

Isoform 1 (identifier: P20851-1) [UniParc]FASTAAdd to basket

This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

« Hide

        10         20         30         40         50
MFFWCACCLM VAWRVSASDA EHCPELPPVD NSIFVAKEVE GQILGTYVCI
60 70 80 90 100
KGYHLVGKKT LFCNASKEWD NTTTECRLGH CPDPVLVNGE FSSSGPVNVS
110 120 130 140 150
DKITFMCNDH YILKGSNRSQ CLEDHTWAPP FPICKSRDCD PPGNPVHGYF
160 170 180 190 200
EGNNFTLGST ISYYCEDRYY LVGVQEQQCV DGEWSSALPV CKLIQEAPKP
210 220 230 240 250
ECEKALLAFQ ESKNLCEAME NFMQQLKESG MTMEELKYSL ELKKAELKAK

LL
Length:252
Mass (Da):28,357
Last modified:February 1, 1991 - v1
Checksum:i0F6CC64067C5E2E7
GO
Isoform 2 (identifier: P20851-2) [UniParc]FASTAAdd to basket

The sequence of this isoform differs from the canonical sequence as follows:
     20-20: Missing.

Note: No experimental confirmation available.
Show »
Length:251
Mass (Da):28,286
Checksum:i2BA0722033999AF4
GO

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_038734102K → Q.1 PublicationCorresponds to variant rs56258224dbSNPEnsembl.1
Natural variantiVAR_012039198P → S.Corresponds to variant rs1803226dbSNPEnsembl.1

Alternative sequence

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Alternative sequenceiVSP_02259420Missing in isoform 2. 1 Publication1

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L11244 mRNA. Translation: AAA35615.1.
L11245 mRNA. Translation: AAA35616.1.
M29964 mRNA. Translation: AAB59520.1.
EF613556 Genomic DNA. Translation: ABQ52216.1.
AL445493 Genomic DNA. Translation: CAH70780.1.
AL445493 Genomic DNA. Translation: CAH70781.1.
CH471100 Genomic DNA. Translation: EAW93504.1.
CH471100 Genomic DNA. Translation: EAW93506.1.
BC005378 mRNA. Translation: AAH05378.1.
CCDSiCCDS1476.1. [P20851-1]
CCDS31005.1. [P20851-2]
PIRiA47107. A34877.
RefSeqiNP_000707.1. NM_000716.3. [P20851-1]
NP_001017364.1. NM_001017364.1. [P20851-2]
NP_001017365.1. NM_001017365.2. [P20851-1]
NP_001017366.1. NM_001017366.2. [P20851-2]
NP_001017367.1. NM_001017367.1. [P20851-1]
XP_005273311.1. XM_005273254.4. [P20851-1]
UniGeneiHs.99886.

Genome annotation databases

EnsembliENST00000243611; ENSP00000243611; ENSG00000123843. [P20851-1]
ENST00000367076; ENSP00000356043; ENSG00000123843. [P20851-2]
ENST00000367078; ENSP00000356045; ENSG00000123843. [P20851-1]
ENST00000391923; ENSP00000375790; ENSG00000123843. [P20851-1]
GeneIDi725.
KEGGihsa:725.
UCSCiuc001hfj.4. human. [P20851-1]

Keywords - Coding sequence diversityi

Alternative splicing, Polymorphism

Cross-referencesi

Web resourcesi

SeattleSNPs

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
L11244 mRNA. Translation: AAA35615.1.
L11245 mRNA. Translation: AAA35616.1.
M29964 mRNA. Translation: AAB59520.1.
EF613556 Genomic DNA. Translation: ABQ52216.1.
AL445493 Genomic DNA. Translation: CAH70780.1.
AL445493 Genomic DNA. Translation: CAH70781.1.
CH471100 Genomic DNA. Translation: EAW93504.1.
CH471100 Genomic DNA. Translation: EAW93506.1.
BC005378 mRNA. Translation: AAH05378.1.
CCDSiCCDS1476.1. [P20851-1]
CCDS31005.1. [P20851-2]
PIRiA47107. A34877.
RefSeqiNP_000707.1. NM_000716.3. [P20851-1]
NP_001017364.1. NM_001017364.1. [P20851-2]
NP_001017365.1. NM_001017365.2. [P20851-1]
NP_001017366.1. NM_001017366.2. [P20851-2]
NP_001017367.1. NM_001017367.1. [P20851-1]
XP_005273311.1. XM_005273254.4. [P20851-1]
UniGeneiHs.99886.

3D structure databases

ProteinModelPortaliP20851.
SMRiP20851.
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi107186. 15 interactors.
STRINGi9606.ENSP00000243611.

PTM databases

iPTMnetiP20851.
PhosphoSitePlusiP20851.

Polymorphism and mutation databases

BioMutaiC4BPB.
DMDMi115213.

Proteomic databases

MaxQBiP20851.
PaxDbiP20851.
PeptideAtlasiP20851.
PRIDEiP20851.

Protocols and materials databases

DNASUi725.
Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000243611; ENSP00000243611; ENSG00000123843. [P20851-1]
ENST00000367076; ENSP00000356043; ENSG00000123843. [P20851-2]
ENST00000367078; ENSP00000356045; ENSG00000123843. [P20851-1]
ENST00000391923; ENSP00000375790; ENSG00000123843. [P20851-1]
GeneIDi725.
KEGGihsa:725.
UCSCiuc001hfj.4. human. [P20851-1]

Organism-specific databases

CTDi725.
DisGeNETi725.
GeneCardsiC4BPB.
HGNCiHGNC:1328. C4BPB.
HPAiHPA051620.
MIMi120831. gene.
neXtProtiNX_P20851.
OpenTargetsiENSG00000123843.
PharmGKBiPA25908.
GenAtlasiSearch...

Phylogenomic databases

eggNOGiKOG4297. Eukaryota.
ENOG410XPJ1. LUCA.
GeneTreeiENSGT00530000063826.
HOGENOMiHOG000032571.
HOVERGENiHBG004922.
InParanoidiP20851.
KOiK04003.
OMAiILKGSNW.
OrthoDBiEOG091G0JTL.
PhylomeDBiP20851.
TreeFamiTF342864.

Enzyme and pathway databases

BioCyciZFISH:ENSG00000123843-MONOMER.
ReactomeiR-HSA-977606. Regulation of Complement cascade.

Miscellaneous databases

GenomeRNAii725.
PROiP20851.
SOURCEiSearch...

Gene expression databases

BgeeiENSG00000123843.
CleanExiHS_C4BPB.
ExpressionAtlasiP20851. baseline and differential.
GenevisibleiP20851. HS.

Family and domain databases

CDDicd00033. CCP. 3 hits.
InterProiIPR000436. Sushi_SCR_CCP_dom.
[Graphical view]
PfamiPF00084. Sushi. 3 hits.
[Graphical view]
SMARTiSM00032. CCP. 3 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 3 hits.
PROSITEiPS50923. SUSHI. 3 hits.
[Graphical view]
ProtoNetiSearch...

Entry informationi

Entry nameiC4BPB_HUMAN
AccessioniPrimary (citable) accession number: P20851
Secondary accession number(s): A5JYP8
, D3DT81, Q5VVR0, Q9BS25
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1991
Last sequence update: February 1, 1991
Last modified: November 30, 2016
This is version 150 of the entry and version 1 of the sequence. [Complete history]
Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

Miscellaneousi

Keywords - Technical termi

Complete proteome, Reference proteome

Documents

  1. Human chromosome 1
    Human chromosome 1: entries, gene names and cross-references to MIM
  2. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  3. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
  5. SIMILARITY comments
    Index of protein domains and families

Similar proteinsi

Links to similar proteins from the UniProt Reference Clusters (UniRef) at 100%, 90% and 50% sequence identity:
100%UniRef100 combines identical sequences and sub-fragments with 11 or more residues from any organism into one UniRef entry.
90%UniRef90 is built by clustering UniRef100 sequences that have at least 90% sequence identity to, and 80% overlap with, the longest sequence (a.k.a seed sequence).
50%UniRef50 is built by clustering UniRef90 seed sequences that have at least 50% sequence identity to, and 80% overlap with, the longest sequence in the cluster.