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25 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ABCC4
ATP binding cassette subfamily C member 4 (PEL blood group)
Chromosome 13 · 13q32.1
NCBI Gene: 10257Ensembl: ENSG00000125257.17HGNC: HGNC:55UniProt: A8K2Q2
242PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transporter
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cAMP transportleukotriene transportexport across plasma membraneprostaglandin transportmental or behavioural disordercataracttrauma complicationlaryngeal disease
✦AI Summary

ABCC4 is an ATP-dependent transporter of the ABC family that actively extrudes diverse endogenous compounds and xenobiotics from cells 1. Its primary substrates include cyclic nucleotides (cAMP and cGMP), bile acids, prostaglandins, urate, and glutathione conjugates such as leukotrienes 23. ABCC4 also transports anticancer, antiviral, and antibiotic drugs, conferring resistance to agents like methotrexate 1. Mechanistically, ABCC4 functions within macromolecular complexes: it physically couples with CFTR in airway epithelial cells to regulate cAMP signaling 4, and PKA activation assembles a PDZ-dependent protein network that stabilizes ABCC4 at the cell surface and optimizes cAMP efflux 5. ABCC4 mediates transport of platelet agonists and antagonists, suggesting relevance to hemostasis and thrombosis 6. Clinically, ABCC4 polymorphisms affect drug resistance; the G187W variant reduces SN-38 resistance 7. Recent evidence identifies ABCC4 as a regulator of LDL cholesterol clearance through cAMP-dependent suppression of PCSK9, linking ABCC4 inhibition to potential atherosclerosis therapeutics 8. Species differences in ABCC4 substrate selectivity—notably human ABCC4 transports arsenic metabolites while mouse Abcc4 does not—may explain toxicokinetic variations between organisms 9.

Sources cited
1
ABCC4 actively extrudes physiological compounds and xenobiotics; transports anticancer, antiviral, and antibiotic drugs
PMID: 11856762
2
ABCC4 transports cyclic nucleotides, bile acids, and mediates cotransport with reduced glutathione
PMID: 12523936
3
ABCC4 transports endogenous molecules including cyclic nucleotides and xenobiotics
PMID: 15364914
4
ABCC4 and CFTR are physically coupled in airway epithelial cells to regulate cAMP-dependent signaling
PMID: 33529712
5
PKA activation assembles a PDZ-dependent ABCC4 macromolecular complex that stabilizes the transporter and optimizes cAMP efflux
PMID: 41339628
6
ABCC4 transports platelet agonists and antagonists, making it a target for cardiovascular disease prevention
PMID: 39195918
7
ABCC4 G187W polymorphism reduces drug resistance to SN-38
PMID: 30634695
8
ABCC4 regulates LDL cholesterol clearance through cAMP-dependent suppression of PCSK9 and LDLR expression
PMID: 41038958
9
Human ABCC4 transports arsenic metabolites while mouse Abcc4 does not, contributing to species differences in arsenic toxicokinetics
PMID: 39313329
Disease Associationsⓘ20
mental or behavioural disorderOpen Targets
0.33Weak
cataractOpen Targets
0.31Weak
trauma complicationOpen Targets
0.30Weak
laryngeal diseaseOpen Targets
0.29Weak
placental retentionOpen Targets
0.27Weak
poisoningOpen Targets
0.26Weak
response to antihypertensive drugOpen Targets
0.26Weak
Tietze syndromeOpen Targets
0.23Weak
angiosarcomaOpen Targets
0.21Weak
blood platelet diseaseOpen Targets
0.18Weak
qualitative platelet defectOpen Targets
0.18Weak
prostate cancerOpen Targets
0.16Weak
Familial prostate cancerOpen Targets
0.14Weak
neoplasmOpen Targets
0.11Weak
colorectal carcinomaOpen Targets
0.10Suggestive
posterior cortical atrophyOpen Targets
0.08Suggestive
sign or symptomOpen Targets
0.08Suggestive
lung cancerOpen Targets
0.08Suggestive
acute myeloid leukemiaOpen Targets
0.07Suggestive
hepatocellular carcinomaOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SLC22A11Protein interaction93%SLC22A12Protein interaction93%CYP7A1Protein interaction91%SLCO1B3Protein interaction87%SNX27Protein interaction80%NHERF1Protein interaction77%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
60%
Brain
52%
Ovary
51%
Heart
32%
Liver
12%
Gene Interaction Network
Click a node to explore
ABCC4SLC22A11SLC22A12CYP7A1SLCO1B3SNX27NHERF1
PROTEIN STRUCTURE
Preparing viewer…
PDB8XOK · 2.84 Å · EM
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.65LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.54 [0.44–0.65]
RankingsWhere ABCC4 stands among ~20K protein-coding genes
  • #1,609of 20,598
    Most Researched242 · top 10%
  • #4,661of 17,882
    Most Constrained (LOEUF)0.65
Genes detectedABCC4
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
Interactions between ABCC4/MRP4 and ABCC7/CFTR in human airway epithelial cells in lung health and disease.
PMID: 33529712
Int J Biochem Cell Biol · 2021
1.00
2
Organic anion transporters in remote sensing and organ crosstalk.
PMID: 39284369
Pharmacol Ther · 2024
0.90
3
Structural insights into human ABCC4-mediated transport of platelet agonist and antagonist.
PMID: 39195918
Nat Cardiovasc Res · 2023
0.80
4
Protein Kinase A and assembly of an ABCC4 protein network.
PMID: 41339628
Nat Commun · 2025
0.70
5
Circ-ABCC4 contributes to prostate cancer progression and radioresistance by mediating miR-1253/SOX4 cascade.
PMID: 36539368
Anticancer Drugs · 2023
0.68