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C4BPA complement component 4 binding protein alpha [ Homo sapiens (human) ]

Gene ID: 722, updated on 3-Apr-2024

Summary

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Official Symbol
C4BPAprovided by HGNC
Official Full Name
complement component 4 binding protein alphaprovided by HGNC
Primary source
HGNC:HGNC:1325
See related
Ensembl:ENSG00000123838 MIM:120830; AllianceGenome:HGNC:1325
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Homo sapiens
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae; Homo
Also known as
PRP; C4BP
Summary
This gene encodes a member of a superfamily of proteins composed predominantly of tandemly arrayed short consensus repeats of approximately 60 amino acids. Along with a single, unique beta-chain, seven identical alpha-chains encoded by this gene assemble into the predominant isoform of C4b-binding protein, a multimeric protein that controls activation of the complement cascade through the classical pathway. The genes encoding both alpha and beta chains are located adjacent to each other on human chromosome 1 in the regulator of complement activation gene cluster. Two pseudogenes of this gene are also found in the cluster. [provided by RefSeq, Jul 2008]
Expression
Biased expression in liver (RPKM 372.5) and lung (RPKM 75.1) See more
Orthologs
all
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Genomic context

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See C4BPA in Genome Data Viewer
Location:
1q32.2
Exon count:
12
Annotation release Status Assembly Chr Location
RS_2023_10 current GRCh38.p14 (GCF_000001405.40) 1 NC_000001.11 (207104233..207144972)
RS_2023_10 current T2T-CHM13v2.0 (GCF_009914755.1) 1 NC_060925.1 (206369472..206410221)
105.20220307 previous assembly GRCh37.p13 (GCF_000001405.25) 1 NC_000001.10 (207277578..207318317)

Chromosome 1 - NC_000001.11Genomic Context describing neighboring genes Neighboring gene NANOG-H3K27ac-H3K4me1 hESC enhancer GRCh37_chr1:207224117-207224687 Neighboring gene H3K27ac-H3K4me1 hESC enhancer GRCh37_chr1:207225911-207226550 Neighboring gene 6-phosphofructo-2-kinase/fructose-2,6-biphosphatase 2 Neighboring gene ReSE screen-validated silencer GRCh37_chr1:207240869-207241100 Neighboring gene YOD1 deubiquitinase Neighboring gene ATAC-STARR-seq lymphoblastoid active region 2439 Neighboring gene complement component 4 binding protein beta Neighboring gene uncharacterized LOC107985251 Neighboring gene Sharpr-MPRA regulatory region 9788 Neighboring gene C4BPA pseudogene 1 Neighboring gene C4BPA pseudogene 3

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: HPA RNA-seq normal tissues
  • Description: RNA-seq was performed of tissue samples from 95 human individuals representing 27 different tissues in order to determine tissue-specificity of all protein-coding genes
  • BioProject: PRJEB4337
  • Publication: PMID 24309898
  • Analysis date: Wed Apr 4 07:08:55 2018

Bibliography

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Related articles in PubMed

  1. Immunoexpression of C4 Binding Protein in Oral Leukoplakia and Oral Squamous Cell Carcinoma. Mashhadiabbas F, et al. Iran J Immunol, 2021 Jun. PMID 34190690
  2. Observation of Complement Protein Gene Expression Before and After Surgery in Opioid-Consuming and Opioid-Naive Patients. Johnson KB, et al. Anesth Analg, 2021 Jan 1. PMID 30169415
  3. Association of single nucleotide polymorphisms in the 5' upstream region of the C4BPA gene with essential hypertension in a northeastern Han Chinese population. Liu X, et al. Mol Med Rep, 2017 Aug. PMID 28627632, Free PMC Article
  4. C4b-binding protein: The good, the bad and the deadly. Novel functions of an old friend. Ermert D, et al. Immunol Lett, 2016 Jan. PMID 26658464
  5. [The early C4d urinary excretion and long-term kidney graft survival in patients treated with tacrolimus and mycophenolate mophetil]. Zwiech R. Pol Merkur Lekarski, 2014 Mar. PMID 24779215

See all (91) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. The Complement System and C4b-Binding Protein: A Focus on the Promise of C4BPalpha as a Biomarker to Predict Clopidogrel Resistance.
    Title: The Complement System and C4b-Binding Protein: A Focus on the Promise of C4BPα as a Biomarker to Predict Clopidogrel Resistance.
  2. Complement Activation-Independent Attenuation of SARS-CoV-2 Infection by C1q and C4b-Binding Protein.
    Title: Complement Activation-Independent Attenuation of SARS-CoV-2 Infection by C1q and C4b-Binding Protein.
  3. Potential biomarkers of spinal dural arteriovenous fistula: C4BPA and C1QA.
    Title: Potential biomarkers of spinal dural arteriovenous fistula: C4BPA and C1QA.
  4. The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity.
    Title: The Hidden Side of Complement Regulator C4BP: Dissection and Evaluation of Its Immunomodulatory Activity.
  5. Immunoexpression of C4 Binding Protein in Oral Leukoplakia and Oral Squamous Cell Carcinoma.
    Title: Immunoexpression of C4 Binding Protein in Oral Leukoplakia and Oral Squamous Cell Carcinoma.
  6. C4b-binding protein alpha-chain enhances antitumor immunity by facilitating the accumulation of tumor-infiltrating lymphocytes in the tumor microenvironment in pancreatic cancer.
    Title: C4b-binding protein α-chain enhances antitumor immunity by facilitating the accumulation of tumor-infiltrating lymphocytes in the tumor microenvironment in pancreatic cancer.
  7. Observation of Complement Protein Gene Expression Before and After Surgery in Opioid-Consuming and Opioid-Naive Patients.
    Title: Observation of Complement Protein Gene Expression Before and After Surgery in Opioid-Consuming and Opioid-Naive Patients.
  8. Fetal lung C4BPA induces p100 processing in human placenta.
    Title: Fetal lung C4BPA induces p100 processing in human placenta.
  9. The authors conclude that Streptococcus pneumoniae PspA and PspC help the pneumococcus to evade complement attack by binding C4BP and so inactivating C4b.
    Title: The Pneumococcal Surface Proteins PspA and PspC Sequester Host C4-Binding Protein To Inactivate Complement C4b on the Bacterial Surface.
  10. The rs73079108 polymorphism in the 5' upstream region of C4BPA was associated with EH, and rs73079108A may be an independent predictor.
    Title: Association of single nucleotide polymorphisms in the 5' upstream region of the C4BPA gene with essential hypertension in a northeastern Han Chinese population.
Submit: New GeneRIF Correction See all GeneRIFs (49)

Phenotypes

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Find tests for this gene in the NIH Genetic Testing Registry (GTR)

Review eQTL and phenotype association data in this region using PheGenI

EBI GWAS Catalog

Description
C4BPB/C4BPA is a new susceptibility locus for venous thrombosis with unknown protein S-independent mechanism: results from genome-wide association and gene expression analyses followed by case-control studies.
EBI GWAS Catalog

Variation

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See variants in ClinVar

See studies and variants in dbVar

See Variation Viewer (GRCh37.p13)

See Variation Viewer (GRCh38)

Genotypes

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Gene Ontology Provided by GOA

Function Evidence Code Pubs
enables RNA binding HDA PubMed 
enables protein binding IPI
Inferred from Physical Interaction
more info
 PubMed 
Process Evidence Code Pubs
involved_in T cell mediated immunity IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in complement activation, classical pathway IEA
Inferred from Electronic Annotation
more info
  
involved_in innate immune response IEA
Inferred from Electronic Annotation
more info
  
involved_in negative regulation of complement activation, classical pathway IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in negative regulation of complement activation, classical pathway IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in positive regulation of protein catabolic process IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in regulation of opsonization IDA
Inferred from Direct Assay
more info
 PubMed 
involved_in response to symbiotic bacterium IDA
Inferred from Direct Assay
more info
 PubMed 
Component Evidence Code Pubs
located_in blood microparticle HDA PubMed 
located_in extracellular region NAS
Non-traceable Author Statement
more info
 PubMed 
located_in extracellular space HDA PubMed 
is_active_in extracellular space IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in extracellular space IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in plasma membrane IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in plasma membrane TAS
Traceable Author Statement
more info
  

General protein information

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Preferred Names
C4b-binding protein alpha chain
Names
proline-rich protein

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

Genomic

  1. NG_029579.1 RefSeqGene

    Range
    4972..45711
    Download
    GenBank, FASTA, Sequence Viewer (Graphics), LRG_547

mRNA and Protein(s)

  1. NM_000715.4 → NP_000706.1  C4b-binding protein alpha chain precursor

    See identical proteins and their annotated locations for NP_000706.1

    Status: REVIEWED

    Source sequence(s)
    AL445493, BC022312, BQ019292
    Consensus CDS
    CCDS1477.1
    UniProtKB/Swiss-Prot
    P04003, Q5VVQ8
    UniProtKB/TrEMBL
    B4E1E1
    Related
    ENSP00000356037.3, ENST00000367070.8
    Conserved Domains (4) summary
    cd00033
    Location:175 → 235
    CCP; Complement control protein (CCP) modules (aka short consensus repeats SCRs or SUSHI repeats) have been identified in several proteins of the complement system
    smart00032
    Location:365 → 422
    CCP; Domain abundant in complement control proteins; SUSHI repeat; short complement-like repeat (SCR)
    PHA02927
    Location:298 → 538
    PHA02927; secreted complement-binding protein; Provisional
    pfam00084
    Location:484 → 538
    Sushi; Sushi repeat (SCR repeat)

RefSeqs of Annotated Genomes: GCF_000001405.40-RS_2023_10

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCh38.p14 Primary Assembly

Genomic

  1. NC_000001.11 Reference GRCh38.p14 Primary Assembly

    Range
    207104233..207144972
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_005273252.5 → XP_005273309.1  C4b-binding protein alpha chain isoform X1

    See identical proteins and their annotated locations for XP_005273309.1

    UniProtKB/Swiss-Prot
    P04003, Q5VVQ8
    UniProtKB/TrEMBL
    B4E1E1
    Conserved Domains (4) summary
    cd00033
    Location:175 → 235
    CCP; Complement control protein (CCP) modules (aka short consensus repeats SCRs or SUSHI repeats) have been identified in several proteins of the complement system
    smart00032
    Location:365 → 422
    CCP; Domain abundant in complement control proteins; SUSHI repeat; short complement-like repeat (SCR)
    PHA02927
    Location:298 → 538
    PHA02927; secreted complement-binding protein; Provisional
    pfam00084
    Location:484 → 538
    Sushi; Sushi repeat (SCR repeat)

  2. XM_005273251.3 → XP_005273308.1  C4b-binding protein alpha chain isoform X1

    See identical proteins and their annotated locations for XP_005273308.1

    UniProtKB/Swiss-Prot
    P04003, Q5VVQ8
    UniProtKB/TrEMBL
    B4E1E1
    Conserved Domains (4) summary
    cd00033
    Location:175 → 235
    CCP; Complement control protein (CCP) modules (aka short consensus repeats SCRs or SUSHI repeats) have been identified in several proteins of the complement system
    smart00032
    Location:365 → 422
    CCP; Domain abundant in complement control proteins; SUSHI repeat; short complement-like repeat (SCR)
    PHA02927
    Location:298 → 538
    PHA02927; secreted complement-binding protein; Provisional
    pfam00084
    Location:484 → 538
    Sushi; Sushi repeat (SCR repeat)

Alternate T2T-CHM13v2.0

Genomic

  1. NC_060925.1 Alternate T2T-CHM13v2.0

    Range
    206369472..206410221
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)

mRNA and Protein(s)

  1. XM_054338582.1 → XP_054194557.1  C4b-binding protein alpha chain isoform X1

  2. XM_054338581.1 → XP_054194556.1  C4b-binding protein alpha chain isoform X1

Nucleotide Protein
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
P04003.2 GenPept UniProtKB/Swiss-Prot:P04003

Gene LinkOut

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