P20851 (C4BPB_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
C4b-binding protein beta chain

Gene names

Name:

C4BPB

Organism

Homo sapiens (Human) [Reference proteome]

Taxonomic identifier

9606 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Primates › Haplorrhini › Simiiformes › Catarrhini › Hominoidea › Hominidae › Homininae › Homo

Protein attributes

Sequence length	252 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

Function	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.
Subunit structure	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.
Subcellular location	Secreted.
Sequence similarities	Contains 3 Sushi (CCP/SCR) domains.

Ontologies

Keywords
Biological process	Complement pathway Immunity Innate immunity
Cellular component	Secreted
Coding sequence diversity	Alternative splicing Polymorphism
Domain	Repeat Signal Sushi
PTM	Disulfide bond Glycoprotein
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	blood coagulation Traceable author statement Ref.1. Source: ProtInc complement activation, classical pathway Inferred from electronic annotation. Source: UniProtKB-KW innate immune response Traceable author statement. Source: Reactome regulation of complement activation Traceable author statement. Source: Reactome
Cellular_component	extracellular region Non-traceable author statement PubMed 14718574. Source: UniProtKB plasma membrane Traceable author statement. Source: Reactome
Complete GO annotation...

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]

Isoform 1 (identifier: P20851-1)

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.

Isoform 2 (identifier: P20851-2)

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

Note: No experimental confirmation available.

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Signal peptide	1 – 17	17
	Chain	18 – 252	235	C4b-binding protein beta chain		PRO_0000005892
Regions
	Domain	21 – 78	58	Sushi 1
	Domain	79 – 136	58	Sushi 2
	Domain	137 – 193	57	Sushi 3
Amino acid modifications
	Glycosylation	64	1	N-linked (GlcNAc...) Ref.7 Ref.8
	Glycosylation	71	1	N-linked (GlcNAc...) Ref.7
	Glycosylation	98	1	N-linked (GlcNAc...) Ref.7 Ref.8 Ref.9
	Glycosylation	117	1	N-linked (GlcNAc...) Potential
	Glycosylation	154	1	N-linked (GlcNAc...) Potential
	Disulfide bond	23 ↔ 63		By similarity
	Disulfide bond	49 ↔ 76		By similarity
	Disulfide bond	81 ↔ 121		By similarity
	Disulfide bond	107 ↔ 134		By similarity
	Disulfide bond	139 ↔ 179		By similarity
	Disulfide bond	165 ↔ 191		By similarity
	Disulfide bond	202		Interchain (with alpha chain) Potential
	Disulfide bond	216		Interchain (with alpha chain) Potential
Natural variations
	Alternative sequence	20	1	Missing in isoform 2.		VSP_022594
	Natural variant	102	1	K → Q. Ref.3 Corresponds to variant rs56258224 [ dbSNP | Ensembl ].		VAR_038734
	Natural variant	198	1	P → S. Corresponds to variant rs1803226 [ dbSNP | Ensembl ].		VAR_012039

Sequences

	Sequence		Length	Mass (Da)
	Isoform 1 [UniParc]. Last modified February 1, 1991. Version 1. Checksum: 0F6CC64067C5E2E7	FASTA	252	28,357
	10 20 30 40 50 60 MFFWCACCLM VAWRVSASDA EHCPELPPVD NSIFVAKEVE GQILGTYVCI KGYHLVGKKT 70 80 90 100 110 120 LFCNASKEWD NTTTECRLGH CPDPVLVNGE FSSSGPVNVS DKITFMCNDH YILKGSNRSQ 130 140 150 160 170 180 CLEDHTWAPP FPICKSRDCD PPGNPVHGYF EGNNFTLGST ISYYCEDRYY LVGVQEQQCV 190 200 210 220 230 240 DGEWSSALPV CKLIQEAPKP ECEKALLAFQ ESKNLCEAME NFMQQLKESG MTMEELKYSL 250 ELKKAELKAK LL
	Isoform 2 [UniParc]. Checksum: 2BA0722033999AF4	FASTA	251	28,286
	10 20 30 40 50 60 MFFWCACCLM VAWRVSASDE HCPELPPVDN SIFVAKEVEG QILGTYVCIK GYHLVGKKTL 70 80 90 100 110 120 FCNASKEWDN TTTECRLGHC PDPVLVNGEF SSSGPVNVSD KITFMCNDHY ILKGSNRSQC 130 140 150 160 170 180 LEDHTWAPPF PICKSRDCDP PGNPVHGYFE GNNFTLGSTI SYYCEDRYYL VGVQEQQCVD 190 200 210 220 230 240 GEWSSALPVC KLIQEAPKPE CEKALLAFQE SKNLCEAMEN FMQQLKESGM TMEELKYSLE 250 LKKAELKAKL L

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"The human C4b-binding protein beta-chain gene." Hillarp A., Pardo-Manuel F., Ruiz R., de Cordoba S., Dahlback B. J. Biol. Chem. 268:15017-15023(1993) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
[2]	"Cloning of cDNA coding for the beta chain of human complement component C4b-binding protein: sequence homology with the alpha chain." Hillarp A., Dahlback B. Proc. Natl. Acad. Sci. U.S.A. 87:1183-1187(1990) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
[3]	SeattleSNPs variation discovery resource Submitted (MAY-2007) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], VARIANT GLN-102.
[4]	"The DNA sequence and biological annotation of human chromosome 1." Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K. , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R. Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[5]	Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C. Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
[6]	"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) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2). Tissue: Skeletal muscle.
[7]	"Screening for N-glycosylated proteins by liquid chromatography mass spectrometry." Bunkenborg J., Pilch B.J., Podtelejnikov A.V., Wisniewski J.R. Proteomics 4:454-465(2004) [PubMed] [Europe PMC] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-64; ASN-71 AND ASN-98, MASS SPECTROMETRY. Tissue: Plasma.
[8]	"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) [PubMed] [Europe PMC] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-64 AND ASN-98, MASS SPECTROMETRY. Tissue: Plasma.
[9]	"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) [PubMed] [Europe PMC] [Abstract] Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-98, MASS SPECTROMETRY. Tissue: Liver.
+	Additional computationally mapped references.

Web resources

SeattleSNPs

Cross-references

Sequence databases
EMBL GenBank DDBJ	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.
IPI	IPI00025862. IPI00555752.
PIR	A34877. A47107.
RefSeq	NP_000707.1. NM_000716.3. NP_001017364.1. NM_001017364.1. NP_001017365.1. NM_001017365.1. NP_001017366.1. NM_001017366.1. NP_001017367.1. NM_001017367.1.
UniGene	Hs.99886.
3D structure databases
ProteinModelPortal	P20851.
ModBase	Search...
Protein-protein interaction databases
STRING	9606.ENSP00000243611.
Polymorphism databases
DMDM	115213.
Proteomic databases
PaxDb	P20851.
PRIDE	P20851.
Protocols and materials databases
DNASU	725.
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENST00000243611; ENSP00000243611; ENSG00000123843. ENST00000367076; ENSP00000356043; ENSG00000123843. ENST00000367078; ENSP00000356045; ENSG00000123843. ENST00000391923; ENSP00000375790; ENSG00000123843.
GeneID	725.
KEGG	hsa:725.
UCSC	uc001hfi.3. human. uc001hfj.3. human.
Organism-specific databases
CTD	725.
GeneCards	GC01P207262.
HGNC	HGNC:1328. C4BPB.
HPA	HPA051620.
MIM	120831. gene.
neXtProt	NX_P20851.
PharmGKB	PA25908.
GenAtlas	Search...
Phylogenomic databases
eggNOG	NOG260856.
HOGENOM	HOG000032571.
HOVERGEN	HBG004922.
InParanoid	P20851.
KO	K04003.
OMA	SSALPVC.
OrthoDB	EOG4RBQK9.
PhylomeDB	P20851.
Enzyme and pathway databases
Pathway_Interaction_DB	hnf3apathway. FOXA1 transcription factor network.
Reactome	REACT_6900. Immune System.
Gene expression databases
ArrayExpress	P20851.
Bgee	P20851.
CleanEx	HS_C4BPB.
Genevestigator	P20851.
GermOnline	ENSG00000123843. Homo sapiens.
Family and domain databases
InterPro	IPR000436. Sushi_SCR_CCP. [Graphical view]
Pfam	PF00084. Sushi. 3 hits. [Graphical view]
SMART	SM00032. CCP. 3 hits. [Graphical view]
SUPFAM	SSF57535. Complement_control_module. 3 hits.
PROSITE	PS50923. SUSHI. 3 hits. [Graphical view]
ProtoNet	Search...
Other
GenomeRNAi	725.
NextBio	2944.
SOURCE	Search...

Entry information

Entry name

C4BPB_HUMAN

Accession

Primary (citable) accession number: P20851
Secondary accession number(s): A5JYP8 , D3DT81, Q5VVR0, Q9BS25

Entry history

Integrated into UniProtKB/Swiss-Prot:	February 1, 1991
Last sequence update:	February 1, 1991
Last modified:	May 1, 2013
This is version 119 of the entry and version 1 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program

Disclaimer

Any 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.