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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ENPP2
ectonucleotide pyrophosphatase/phosphodiesterase 2
Chromosome 8 · 8q24.12
NCBI Gene: 5168Ensembl: ENSG00000136960.13HGNC: HGNC:3357UniProt: E7EUF1
206PubMed Papers
20Diseases
1Drugs
0Pathogenic Variants
RESEARCH IMPACT
Trending
CLINICAL
Clinical Trials
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
zinc ion bindingsphingolipid catabolic processregulation of cell migrationphosphatidylcholine catabolic processAbnormality of the skeletal systemidiopathic pulmonary fibrosisdiverticular diseaseabdominal abscess
✦AI Summary

ENPP2 (ectonucleotide pyrophosphatase/phosphodiesterase 2), also known as autotaxin, functions as a secreted lysophospholipase D that hydrolyzes lysophosphatidylcholine to produce lysophosphatidic acid (LPA), a critical signaling molecule 1. The enzyme's primary mechanism involves LPA production, which activates six distinct G protein-coupled receptors to regulate multiple cellular processes including angiogenesis, neural development, cell migration, and proliferation 12. ENPP2 is essential for embryonic development, as knockout mice exhibit intrauterine death due to vascular defects and impaired neural development 2. The protein demonstrates significant disease relevance, with altered expression associated with various pathological conditions. In chr8 lymphocytic leukemia, ENPP2 promotes cancer progression through the AMPK/SREBP1/FAS lipid metabolism pathway 3. In reproductive health, defective ENPP2-LPA signaling increases miscarriage risk by restricting decidual macrophage residence through impaired autophagy 4. Additionally, ENPP2 serves as a prognostic biomarker in hepatocellular carcinoma, where high expression correlates with poor outcomes 5. Clinical significance includes its potential as a therapeutic target, with ENPP2 inhibitors showing promise in treating autoimmune diseases and various cancers 63.

Sources cited
1
ENPP2 is a secreted lysophospholipase D that hydrolyzes lysophosphatidylcholine into LPA
PMID: 26391552
2
ENPP2 is essential for embryonic development and vascular formation
PMID: 23022664
3
ENPP2 promotes chronic lymphocytic leukemia progression via lipid metabolism pathways
PMID: 39731014
4
Defective ENPP2-LPA signaling increases miscarriage risk
PMID: 35220880
5
High ENPP2 expression is associated with poor prognosis in hepatocellular carcinoma
PMID: 37543832
6
ENPP2 inhibition blocks cytokine production in autoimmune diseases
PMID: 34717796
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.54Moderate
idiopathic pulmonary fibrosisOpen Targets
0.35Weak
diverticular diseaseOpen Targets
0.31Weak
abdominal abscessOpen Targets
0.31Weak
multiple myelomaOpen Targets
0.29Weak
Abnormal erythrocyte morphologyOpen Targets
0.29Weak
neurodegenerative diseaseOpen Targets
0.27Weak
HypocalcemiaOpen Targets
0.26Weak
exostosisOpen Targets
0.18Weak
alcohol drinkingOpen Targets
0.14Weak
neoplasmOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.11Weak
breast cancerOpen Targets
0.11Weak
cancerOpen Targets
0.11Weak
systemic sclerodermaOpen Targets
0.10Weak
melanomaOpen Targets
0.10Weak
non-alcoholic fatty liver diseaseOpen Targets
0.10Suggestive
COVID-19Open Targets
0.10Suggestive
breast angiosarcomaOpen Targets
0.09Suggestive
cirrhosis of liverOpen Targets
0.09Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets1
ZIRITAXESTATPhase III
Autotaxin inhibitor
idiopathic pulmonary fibrosis
Related Genes
GDPD1Protein interaction99%GDPD3Protein interaction99%LPAR1Protein interaction92%LPAR2Protein interaction80%LPAR3Protein interaction80%LPAR4Protein interaction76%
Tissue Expression6 tissues
Brain
100%
Lung
13%
Heart
8%
Ovary
7%
Bone Marrow
6%
Liver
4%
Gene Interaction Network
Click a node to explore
ENPP2GDPD1GDPD3LPAR1LPAR2LPAR3LPAR4
PROTEIN STRUCTURE
Preparing viewer…
PDB4ZG7 · 1.75 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.64LoF Tolerant
pLIⓘ
0.04Tolerant
Observed/Expected LoF0.45 [0.32–0.64]
RankingsWhere ENPP2 stands among ~20K protein-coding genes
  • #2,027of 20,598
    Most Researched206 · top 10%
  • #4,561of 17,882
    Most Constrained (LOEUF)0.64
Genes detectedENPP2
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
Purine Release, Metabolism, and Signaling in the Inflammatory Response.
PMID: 30676821
Annu Rev Immunol · 2019
1.00
2
A defective lysophosphatidic acid-autophagy axis increases miscarriage risk by restricting decidual macrophage residence.
PMID: 35220880
Autophagy · 2022
0.90
3
ENPP2 promotes progression and lipid accumulation via AMPK/SREBP1/FAS pathway in chronic lymphocytic leukemia.
PMID: 39731014
Cell Mol Biol Lett · 2024
0.80
4
PMID: 34769391
Int J Mol Sci · 2021
0.72
5
Understanding the Multifaceted Role of Ectonucleotide Pyrophosphatase/Phosphodiesterase 2 (ENPP2) and its Altered Behaviour in Human Diseases.
PMID: 26391552
Curr Mol Med · 2015
0.70