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P04003

- C4BPA_HUMAN

UniProt

P04003 - C4BPA_HUMAN

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Protein
C4b-binding protein alpha chain
Gene
C4BPA, C4BP
Organism
Homo sapiens (Human)
Status
Reviewed - 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. Alpha chain binds C4b. It interacts also with anticoagulant protein S and with serum amyloid P component.

GO - Molecular functioni

  1. poly(A) RNA binding Source: UniProtKB
  2. protein binding Source: IntAct

GO - Biological processi

  1. complement activation, classical pathway Source: UniProtKB-KW
  2. innate immune response Source: Reactome
  3. negative regulation of complement activation, classical pathway Source: BHF-UCL
  4. positive regulation of protein catabolic process Source: BHF-UCL
  5. regulation of complement activation Source: Reactome
  6. regulation of opsonization Source: BHF-UCL
Complete GO annotation...

Keywords - Biological processi

Complement pathway, Immunity, Innate immunity

Enzyme and pathway databases

ReactomeiREACT_118707. Regulation of Complement cascade.

Names & Taxonomyi

Protein namesi
Recommended name:
C4b-binding protein alpha chain
Short name:
C4bp
Alternative name(s):
Proline-rich protein
Short name:
PRP
Gene namesi
Name:C4BPA
Synonyms:C4BP
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 1

Organism-specific databases

HGNCiHGNC:1325. C4BPA.

Subcellular locationi

GO - Cellular componenti

  1. blood microparticle Source: UniProt
  2. extracellular region Source: UniProtKB
  3. extracellular space Source: BHF-UCL
  4. other organism cell Source: BHF-UCL
  5. plasma membrane Source: Reactome
Complete GO annotation...

Keywords - Cellular componenti

Secreted

Pathology & Biotechi

Organism-specific databases

PharmGKBiPA25905.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Signal peptidei1 – 48481 Publication
Add
BLAST
Chaini49 – 597549C4b-binding protein alpha chain
PRO_0000005888Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Disulfide bondi50 ↔ 961 Publication
Disulfide bondi81 ↔ 1081 Publication
Disulfide bondi113 ↔ 1541 Publication
Disulfide bondi140 ↔ 1701 Publication
Disulfide bondi175 ↔ 217 By similarity
Disulfide bondi203 ↔ 234 By similarity
Glycosylationi221 – 2211N-linked (GlcNAc...)3 Publications
Disulfide bondi239 ↔ 281 By similarity
Disulfide bondi267 ↔ 294 By similarity
Disulfide bondi299 ↔ 348 By similarity
Disulfide bondi332 ↔ 360 By similarity
Disulfide bondi365 ↔ 409 By similarity
Disulfide bondi399 ↔ 422 By similarity
Disulfide bondi426 ↔ 468 By similarity
Disulfide bondi454 ↔ 480 By similarity
Disulfide bondi484 ↔ 525 By similarity
Glycosylationi506 – 5061N-linked (GlcNAc...)4 Publications
Disulfide bondi511 ↔ 538 By similarity
Glycosylationi528 – 5281N-linked (GlcNAc...)2 Publications
Disulfide bondi546 – 546Interchain (with beta chain) Reviewed prediction
Disulfide bondi558 – 558Interchain (with beta chain) Reviewed prediction

Keywords - PTMi

Disulfide bond, Glycoprotein

Proteomic databases

MaxQBiP04003.
PaxDbiP04003.
PeptideAtlasiP04003.
PRIDEiP04003.

PTM databases

PhosphoSiteiP04003.

Expressioni

Tissue specificityi

Chylomicrons in the plasma.

Gene expression databases

ArrayExpressiP04003.
BgeeiP04003.
CleanExiHS_C4BPA.
GenevestigatoriP04003.

Organism-specific databases

HPAiHPA000926.
HPA001578.

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.

Binary interactionsi

WithEntry#Exp.IntActNotes
arp4P130505EBI-978348,EBI-978341From a different organism.

Protein-protein interaction databases

BioGridi107183. 8 interactions.
IntActiP04003. 6 interactions.
STRINGi9606.ENSP00000356037.

Structurei

Secondary structure

Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Beta strandi85 – 873
Beta strandi98 – 1036
Beta strandi108 – 1103
Beta strandi131 – 1333
Beta strandi158 – 1603
Helixi547 – 5559
Beta strandi558 – 5614
Helixi562 – 58928
Helixi591 – 5966

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2A55NMR-A49-172[»]
4B0FX-ray2.80A/B/C/D/E/F/G540-597[»]
ProteinModelPortaliP04003.
SMRiP04003. Positions 49-597.

Miscellaneous databases

EvolutionaryTraceiP04003.

Family & Domainsi

Domains and Repeats

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Domaini49 – 11062Sushi 1
Add
BLAST
Domaini111 – 17262Sushi 2
Add
BLAST
Domaini173 – 23664Sushi 3
Add
BLAST
Domaini237 – 29660Sushi 4
Add
BLAST
Domaini297 – 36266Sushi 5
Add
BLAST
Domaini363 – 42462Sushi 6
Add
BLAST
Domaini425 – 48258Sushi 7
Add
BLAST
Domaini483 – 54058Sushi 8
Add
BLAST

Sequence similaritiesi

Keywords - Domaini

Repeat, Signal, Sushi

Phylogenomic databases

eggNOGiNOG150577.
HOGENOMiHOG000015324.
HOVERGENiHBG004657.
InParanoidiP04003.
KOiK04002.
OMAiVENETIG.
OrthoDBiEOG725DH2.
PhylomeDBiP04003.
TreeFamiTF334137.

Family and domain databases

InterProiIPR000436. Sushi_SCR_CCP.
[Graphical view]
PfamiPF00084. Sushi. 8 hits.
[Graphical view]
SMARTiSM00032. CCP. 8 hits.
[Graphical view]
SUPFAMiSSF57535. SSF57535. 8 hits.
PROSITEiPS50923. SUSHI. 8 hits.
[Graphical view]

Sequencei

Sequence statusi: Complete.

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

P04003-1 [UniParc]FASTAAdd to Basket

« Hide

MHPPKTPSGA LHRKRKMAAW PFSRLWKVSD PILFQMTLIA ALLPAVLGNC    50
GPPPTLSFAA PMDITLTETR FKTGTTLKYT CLPGYVRSHS TQTLTCNSDG 100
EWVYNTFCIY KRCRHPGELR NGQVEIKTDL SFGSQIEFSC SEGFFLIGST 150
TSRCEVQDRG VGWSHPLPQC EIVKCKPPPD IRNGRHSGEE NFYAYGFSVT 200
YSCDPRFSLL GHASISCTVE NETIGVWRPS PPTCEKITCR KPDVSHGEMV 250
SGFGPIYNYK DTIVFKCQKG FVLRGSSVIH CDADSKWNPS PPACEPNSCI 300
NLPDIPHASW ETYPRPTKED VYVVGTVLRY RCHPGYKPTT DEPTTVICQK 350
NLRWTPYQGC EALCCPEPKL NNGEITQHRK SRPANHCVYF YGDEISFSCH 400
ETSRFSAICQ GDGTWSPRTP SCGDICNFPP KIAHGHYKQS SSYSFFKEEI 450
IYECDKGYIL VGQAKLSCSY SHWSAPAPQC KALCRKPELV NGRLSVDKDQ 500
YVEPENVTIQ CDSGYGVVGP QSITCSGNRT WYPEVPKCEW ETPEGCEQVL 550
TGKRLMQCLP NPEDVKMALE VYKLSLEIEQ LELQRDSARQ STLDKEL 597
Length:597
Mass (Da):67,033
Last modified:October 1, 1993 - v2
Checksum:i67E03F2EA85A16DD
GO

Natural variant

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Natural varianti4 – 41P → Q.
Corresponds to variant rs55867570 [ dbSNP | Ensembl ].
VAR_061123
Natural varianti60 – 601A → V.
Corresponds to variant rs17020956 [ dbSNP | Ensembl ].
VAR_048815
Natural varianti240 – 2401R → H.
Corresponds to variant rs45574833 [ dbSNP | Ensembl ].
VAR_061124
Natural varianti300 – 3001I → T.
Corresponds to variant rs4844573 [ dbSNP | Ensembl ].
VAR_024420
Natural varianti357 – 3571Y → H.
VAR_001978
Natural varianti473 – 4731W → L.
Corresponds to variant rs1801341 [ dbSNP | Ensembl ].
VAR_012038

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
M31452 mRNA. Translation: AAA36507.1.
M62486
, M62475, M62476, M62477, M62478, M62479, M62480, M62481, M62482, M62484, M62485 Genomic DNA. Translation: AAA36506.1.
AK313164 mRNA. Translation: BAG35982.1.
CH471100 Genomic DNA. Translation: EAW93502.1.
CH471100 Genomic DNA. Translation: EAW93503.1.
BC022312 mRNA. Translation: AAH22312.1.
X07853 mRNA. Translation: CAA30701.1.
X04284 Genomic DNA. Translation: CAB51244.1.
X04296 Genomic DNA. Translation: CAA27839.1.
X02865 mRNA. Translation: CAA26617.1.
CCDSiCCDS1477.1.
PIRiA33568. NBHUC4.
RefSeqiNP_000706.1. NM_000715.3.
XP_005273308.1. XM_005273251.1.
XP_005273309.1. XM_005273252.2.
UniGeneiHs.1012.

Genome annotation databases

EnsembliENST00000367070; ENSP00000356037; ENSG00000123838.
GeneIDi722.
KEGGihsa:722.
UCSCiuc001hfo.3. human.

Polymorphism databases

DMDMi416733.

Keywords - Coding sequence diversityi

Polymorphism

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
M31452 mRNA. Translation: AAA36507.1 .
M62486
, M62475 , M62476 , M62477 , M62478 , M62479 , M62480 , M62481 , M62482 , M62484 , M62485 Genomic DNA. Translation: AAA36506.1 .
AK313164 mRNA. Translation: BAG35982.1 .
CH471100 Genomic DNA. Translation: EAW93502.1 .
CH471100 Genomic DNA. Translation: EAW93503.1 .
BC022312 mRNA. Translation: AAH22312.1 .
X07853 mRNA. Translation: CAA30701.1 .
X04284 Genomic DNA. Translation: CAB51244.1 .
X04296 Genomic DNA. Translation: CAA27839.1 .
X02865 mRNA. Translation: CAA26617.1 .
CCDSi CCDS1477.1.
PIRi A33568. NBHUC4.
RefSeqi NP_000706.1. NM_000715.3.
XP_005273308.1. XM_005273251.1.
XP_005273309.1. XM_005273252.2.
UniGenei Hs.1012.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
2A55 NMR - A 49-172 [» ]
4B0F X-ray 2.80 A/B/C/D/E/F/G 540-597 [» ]
ProteinModelPortali P04003.
SMRi P04003. Positions 49-597.
ModBasei Search...
MobiDBi Search...

Protein-protein interaction databases

BioGridi 107183. 8 interactions.
IntActi P04003. 6 interactions.
STRINGi 9606.ENSP00000356037.

PTM databases

PhosphoSitei P04003.

Polymorphism databases

DMDMi 416733.

Proteomic databases

MaxQBi P04003.
PaxDbi P04003.
PeptideAtlasi P04003.
PRIDEi P04003.

Protocols and materials databases

DNASUi 722.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000367070 ; ENSP00000356037 ; ENSG00000123838 .
GeneIDi 722.
KEGGi hsa:722.
UCSCi uc001hfo.3. human.

Organism-specific databases

CTDi 722.
GeneCardsi GC01P207277.
HGNCi HGNC:1325. C4BPA.
HPAi HPA000926.
HPA001578.
MIMi 120830. gene.
neXtProti NX_P04003.
PharmGKBi PA25905.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG150577.
HOGENOMi HOG000015324.
HOVERGENi HBG004657.
InParanoidi P04003.
KOi K04002.
OMAi VENETIG.
OrthoDBi EOG725DH2.
PhylomeDBi P04003.
TreeFami TF334137.

Enzyme and pathway databases

Reactomei REACT_118707. Regulation of Complement cascade.

Miscellaneous databases

EvolutionaryTracei P04003.
GenomeRNAii 722.
NextBioi 2938.
PROi P04003.
SOURCEi Search...

Gene expression databases

ArrayExpressi P04003.
Bgeei P04003.
CleanExi HS_C4BPA.
Genevestigatori P04003.

Family and domain databases

InterProi IPR000436. Sushi_SCR_CCP.
[Graphical view ]
Pfami PF00084. Sushi. 8 hits.
[Graphical view ]
SMARTi SM00032. CCP. 8 hits.
[Graphical view ]
SUPFAMi SSF57535. SSF57535. 8 hits.
PROSITEi PS50923. SUSHI. 8 hits.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Molecular cloning of the cDNA coding for proline-rich protein (PRP): identity of PRP as C4b-binding protein."
    Matsuguchi T., Okamura S., Aso T., Sata T., Niho Y.
    Biochem. Biophys. Res. Commun. 165:138-144(1989) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA].
    Tissue: Liver.
  2. "Genomic organization of the alpha chain of the human C4b-binding protein gene."
    Aso T., Okamura S., Matsuguchi T., Sakamoto N., Sata T., Niho Y.
    Biochem. Biophys. Res. Commun. 174:222-227(1991) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA / MRNA].
  3. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
    Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
    , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
    Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
    Tissue: Lung.
  4. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  5. "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].
    Tissue: Lung.
  6. "Derivation of the sequence of the signal peptide in human C4b-binding protein and interspecies cross-hybridisation of the C4bp cDNA sequence."
    Lintin S.J., Lewin A.R., Reid K.B.M.
    FEBS Lett. 232:328-332(1988) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 9-81.
  7. "Studies on the structure of the human C4b-binding protein gene."
    Lintin S.J., Reid K.B.M.
    FEBS Lett. 204:77-81(1986) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA] OF 203-288.
  8. "Molecular cloning and characterization of the cDNA coding for C4b-binding protein, a regulatory protein of the classical pathway of the human complement system."
    Chung L.P., Bentley D.R., Reid K.B.M.
    Biochem. J. 230:133-141(1985) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] OF 80-597.
  9. "Amino acid sequence studies of human C4b-binding protein: N-terminal sequence analysis and alignment of the fragments produced by limited proteolysis with chymotrypsin and the peptides produced by cyanogen bromide treatment."
    Chung L.P., Gagnon J., Reid K.B.M.
    Mol. Immunol. 22:427-435(1985) [PubMed] [Europe PMC] [Abstract]
    Cited for: PROTEIN SEQUENCE OF 49-88.
  10. "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-506 AND ASN-528.
    Tissue: Plasma.
  11. "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-221 AND ASN-506.
    Tissue: Plasma.
  12. "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-221 AND ASN-506.
    Tissue: Liver.
  13. "Visualization of human C4b-binding protein and its complexes with vitamin K-dependent protein S and complement protein C4b."
    Dahlback B., Smith C.A., Mueller-Eberhard H.J.
    Proc. Natl. Acad. Sci. U.S.A. 80:3461-3465(1983) [PubMed] [Europe PMC] [Abstract]
    Cited for: STRUCTURE BY ELECTRON MICROSCOPY, LIGAND-BINDING.
  14. "Human C4b-binding protein, structural basis for interaction with streptococcal M protein, a major bacterial virulence factor."
    Jenkins H.T., Mark L., Ball G., Persson J., Lindahl G., Uhrin D., Blom A.M., Barlow P.N.
    J. Biol. Chem. 281:3690-3697(2006) [PubMed] [Europe PMC] [Abstract]
    Cited for: STRUCTURE BY NMR OF 49-172, DISULFIDE BONDS.

Entry informationi

Entry nameiC4BPA_HUMAN
AccessioniPrimary (citable) accession number: P04003
Secondary accession number(s): Q5VVQ8
Entry historyi
Integrated into UniProtKB/Swiss-Prot: October 23, 1986
Last sequence update: October 1, 1993
Last modified: September 3, 2014
This is version 147 of the entry and version 2 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

Caution

It is uncertain whether Met-1 or Met-17 is the initiator.

Keywords - Technical termi

3D-structure, Complete proteome, Direct protein sequencing, 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  6. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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