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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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C4BPA
complement component 4 binding protein alpha
Chromosome 1 · 1q32.2
NCBI Gene: 722Ensembl: ENSG00000123838.12HGNC: HGNC:1325UniProt: P04003
95PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
regulation of opsonizationGO:0005615blood microparticleresponse to symbiotic bacteriumvenous thromboembolismdengue diseaseThromboembolismdeep vein thrombosis
✦AI Summary

C4BPA encodes the alpha chain of complement component 4 binding protein (C4BP), a hepatic-derived plasma glycoprotein that negatively regulates the classical complement pathway 1. The protein functions as a cofactor for C3b/C4b inactivator, facilitating hydrolysis of complement fragment C4b and accelerating degradation of the C3 convertase complex C4bC2a 1. C4BPA expression is controlled by the hepatic transcription factor HNF1 through a TATA-less promoter mechanism 1. During acute phase responses, C4BPA expression is differentially modulated by cytokines (IL-6, IL-1β, IFN-γ upregulate; TNF-α downregulates), enabling dynamic adjustment of complement regulation 2. Beyond complement control, emerging evidence implicates C4BPA in pathological inflammation and malignancy. C4BPA is upregulated in spinal dural arteriovenous fistula cerebrospinal fluid (area under ROC curve 0.86) and correlates with acute inflammatory response activation 3. Genetically validated studies identify C4BPA as a druggable target in non-small cell lung cancer, promoting progression via the CCL8 inflammatory chemokine axis 4, and in delirium, where C4BPA explains 16.7% of aging-related risk 5. In gastric cancer, tumor-derived C4BPA drives disease progression by promoting M2-like macrophage polarization through the C5a-C5aR1-STAT3 immunomodulatory pathway 6. These findings position C4BPA as both a complement regulator and inflammatory oncology target.

Sources cited
1
C4BP controls classical complement pathway activation; C4BPA is hepatically expressed; HNF1 transcription factor controls C4BPA promoter activity
PMID: 7772049
2
C4BPA expression is modulated by acute phase cytokines (IL-6, IL-1β, IFN-γ upregulate; TNF-α downregulates)
PMID: 7561114
3
C4BPA is overexpressed in cerebrospinal fluid of SDAVF patients with diagnostic ROC-AUC of 0.86; associated with complement activation and acute inflammatory response
PMID: 35733178
4
C4BPA is genetically validated as a risk factor for NSCLC; promotes progression through CCL8 inflammatory chemokine signaling axis
PMID: 41053817
5
C4BPA identified as druggable target for delirium; explains 16.7% of aging-related delirium risk through mediation analysis
PMID: 40023257
6
Tumor-derived C4BPA promotes gastric cancer progression by driving M2-like macrophage polarization via C5a-C5aR1-STAT3 axis
PMID: 41237697
7
C4BPA identified as rectal cancer-specific non-canonical driver gene
PMID: 40770356
8
C4BPA expression affected by PPP1R1B-USF2 regulatory axis in intrahepatic cholangiocarcinoma
PMID: 38924592
Disease Associationsⓘ20
venous thromboembolismOpen Targets
0.43Moderate
dengue diseaseOpen Targets
0.37Weak
ThromboembolismOpen Targets
0.35Weak
deep vein thrombosisOpen Targets
0.27Weak
Alzheimer diseaseOpen Targets
0.26Weak
neurodegenerative diseaseOpen Targets
0.25Weak
Parkinson diseaseOpen Targets
0.24Weak
lysosomal storage diseaseOpen Targets
0.24Weak
multiple sclerosisOpen Targets
0.24Weak
Creutzfeldt Jacob DiseaseOpen Targets
0.11Weak
scrapieOpen Targets
0.11Weak
variant Creutzfeldt-Jakob diseaseOpen Targets
0.10Suggestive
prion diseaseOpen Targets
0.10Suggestive
breast cancerOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.09Suggestive
infectionOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
Infantile dystonia-parkinsonismOpen Targets
0.07Suggestive
parkinsonism-dystonia, infantileOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PRB1Protein interaction100%PRB3Protein interaction100%CRPProtein interaction99%C4AProtein interaction98%C4BProtein interaction98%C4B_2Protein interaction98%
Tissue Expression6 tissues
Liver
100%
Lung
5%
Ovary
0%
Brain
0%
Heart
0%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
C4BPAPRB1PRB3CRPC4AC4BC4B_2
PROTEIN STRUCTURE
Preparing viewer…
PDB5I0Q · 2.29 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.72LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.50 [0.35–0.72]
RankingsWhere C4BPA stands among ~20K protein-coding genes
  • #5,019of 20,598
    Most Researched95 · top quartile
  • #5,514of 17,882
    Most Constrained (LOEUF)0.72
Genes detectedC4BPA
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Expression of the human gene coding for the alpha-chain of C4b-binding protein, C4BPA, is controlled by an HNF1-dependent hepatic-specific promoter.
PMID: 7772049
Biochem J · 1995
1.00
2
Identification of C4BPA as a genetically informed drug target in NSCLC: an integrative single-cell and multi-omics study based on the druggable genes.
PMID: 41053817
Hum Genomics · 2025
0.90
3
Potential biomarkers of spinal dural arteriovenous fistula: C4BPA and C1QA.
PMID: 35733178
J Neuroinflammation · 2022
0.80
4
Novel drug targets for delirium based on genetic causality.
PMID: 40023257
J Affect Disord · 2025
0.70
5
Isoforms of human C4b-binding protein. II. Differential modulation of the C4BPA and C4BPB genes by acute phase cytokines.
PMID: 7561114
J Immunol · 1995
0.60