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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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PAQR4
progestin and adipoQ receptor family member 4
Chromosome 16 · 16p13.3
NCBI Gene: 124222Ensembl: ENSG00000162073.14HGNC: HGNC:26386UniProt: I3L1A2
21PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
molecular adaptor activityprotein stabilizationregulation of ceramide biosynthetic processGolgi membraneneoplasmnon-small cell lung carcinomacancerhepatocellular carcinoma
✦AI Summary

PAQR4 is a seven-transmembrane orphan receptor that primarily functions in regulating cellular ceramide homeostasis and adipose tissue metabolism. Mechanistically, PAQR4 mediates the stability and activity of ceramide synthases CERS2 and CERS5, controlling de novo ceramide biosynthesis 1. In metabolic contexts, PAQR4 overexpression in adipocytes causes ceramide accumulation, impairing adipogenesis and triggering adipocyte de-differentiation, leading to lipodystrophy, hyperglycemia, and hyperinsulinemia; conversely, adipocyte-specific PAQR4 deletion improves glucose homeostasis during diet-induced obesity 1. Beyond metabolism, PAQR4 is significantly upregulated across multiple cancer types and functions as an oncogene. High PAQR4 expression correlates with poor prognosis, advanced tumor stage, and altered immune microenvironments in bladder, renal, and hepatocellular carcinomas 2. In breast cancer, PAQR4 promotes proliferation by reducing CDK4 protein degradation through decreased polyubiquitination 3. PAQR4 also participates in chemotherapy resistance through multiple signaling pathways and influences cellular senescence and immune evasion 4. Clinically, PAQR4 represents a promising therapeutic target for both metabolic disorders and malignancies, with potential as a prognostic biomarker across cancer types 5.

Sources cited
1
PAQR4 regulates adipocyte ceramide levels by mediating CERS2 and CERS5 stability; overexpression causes lipodystrophy and metabolic dysfunction; deletion improves glucose homeostasis
PMID: 38961186
2
PAQR4 is upregulated in multiple cancers, correlates with poor prognosis and advanced stage, associates with altered immune infiltration and tumor immunity
PMID: 36481756
3
PAQR4 promotes breast cancer proliferation by reducing CDK4 protein degradation and polyubiquitination
PMID: 29228296
4
PAQR4 is a senescence-related gene linked to poor prognosis, immune evasion, and metastasis in bladder urothelial carcinoma
PMID: 40978552
5
PAQR4 is a seven-transmembrane protein that regulates ceramide homeostasis and plays roles in cancer, metabolic diseases, and fertility
PMID: 40194713
Disease Associationsⓘ20
neoplasmOpen Targets
0.10Suggestive
non-small cell lung carcinomaOpen Targets
0.09Suggestive
cancerOpen Targets
0.08Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
breast cancerOpen Targets
0.03Suggestive
gastric cancerOpen Targets
0.02Suggestive
bladder transitional cell carcinomaOpen Targets
0.02Suggestive
uterine leiomyosarcomaOpen Targets
0.02Suggestive
posterior cortical atrophyOpen Targets
0.02Suggestive
urinary bladder carcinomaOpen Targets
0.02Suggestive
colorectal cancerOpen Targets
0.02Suggestive
breast carcinomaOpen Targets
0.01Suggestive
prostate cancerOpen Targets
0.01Suggestive
lung adenocarcinomaOpen Targets
0.01Suggestive
pancreatic adenocarcinomaOpen Targets
0.01Suggestive
obesityOpen Targets
0.01Suggestive
ovarian cancerOpen Targets
0.01Suggestive
small cell lung carcinomaOpen Targets
0.01Suggestive
acute lymphoblastic leukemiaOpen Targets
0.01Suggestive
nasopharyngeal carcinomaOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CPN2Shared pathway100%TELO2Shared pathway100%TSPAN1Shared pathway100%RPAP3Shared pathway100%TTI2Shared pathway100%NAA30Shared pathway100%
Tissue Expression6 tissues
Brain
100%
Bone Marrow
28%
Lung
11%
Heart
9%
Liver
3%
Ovary
1%
Gene Interaction Network
Click a node to explore
PAQR4CPN2TELO2TSPAN1RPAP3TTI2NAA30
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8N4S7
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.71LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.24 [0.91–1.71]
RankingsWhere PAQR4 stands among ~20K protein-coding genes
  • #13,976of 20,598
    Most Researched21
  • #16,137of 17,882
    Most Constrained (LOEUF)1.71
Genes detectedPAQR4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PAQR4 regulates adipocyte function and systemic metabolic health by mediating ceramide levels.
PMID: 38961186
Nat Metab · 2024
1.00
2
PAQR4: From spatial regulation of cell signaling to physiological homeostasis and diseases.
PMID: 40194713
Biochim Biophys Acta Rev Cancer · 2025
0.90
3
PAQR4: A Critical Senescence-Related Gene Influencing Immune Evasion and Metastasis in Bladder Urothelial Carcinoma.
PMID: 40978552
Hum Mutat · 2025
0.80
4
PAQR4 oncogene: a novel target for cancer therapy.
PMID: 38767705
Med Oncol · 2024
0.70
5
Pan-cancer analysis of the prognostic and immunological role of PAQR4.
PMID: 36481756
Sci Rep · 2022
0.60