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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ENTPD5
ectonucleoside triphosphate diphosphohydrolase 5 (inactive)
Chromosome 14 · 14q24.3
NCBI Gene: 957Ensembl: ENSG00000187097.13HGNC: HGNC:3367UniProt: G3V4I0
37PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Oncogene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
ADP phosphatase activityUDP phosphatase activityIDP phosphatase activityGDP phosphatase activityfamilial steroid-resistant nephrotic syndrome with sensorineural deafnessmusculoskeletal system diseasespondylolisthesisAbnormality of the skeletal system
✦AI Summary

ENTPD5 is an endoplasmic reticulum (ER)-localized nucleotidase that hydrolyzes nucleoside diphosphates, particularly GDP, IDP, and UDP 1. Its primary function involves regulating protein N-glycosylation in the ER by hydrolyzing UDP, which acts as a feedback inhibitor of UDP-glucose:glycoprotein glucosyltransferases 1. This activity promotes protein reglucosylation, essential for proper glycoprotein folding and ER quality control 2. ENTPD5 maintains the calnexin/calreticulin cycle, affecting integrin expression and cellular adhesion properties 2. Beyond ER functions, ENTPD5 also hydrolyzes extracellular ATP to ADP, activating purinergic signaling pathways that regulate hepatic glucose and lipid metabolism 3. Clinically, ENTPD5 shows significant disease relevance as it is overexpressed in multiple cancers including ovarian, pancreatic, lung, and breast cancers 452. In epithelial ovarian cancer, ENTPD5 overexpression correlates with poor survival and promotes tumor growth and metastasis through the GRP78/p-eIF-2α/CHOP pathway 5. The enzyme also plays protective roles in diabetic kidney disease by alleviating ER stress-associated renal injury 1. These diverse functions establish ENTPD5 as both a metabolic regulator and potential therapeutic target in cancer treatment 6.

Sources cited
1
ENTPD5 hydrolyzes UDP in the ER and regulates protein N-glycosylation
PMID: 36849424
2
ENTPD5 controls the calnexin/calreticulin cycle and affects integrin expression
PMID: 37563605
3
ENTPD5 hydrolyzes extracellular ATP to regulate hepatic metabolism
PMID: 40788055
4
ENTPD5 is highly expressed in epithelial ovarian cancer
PMID: 33963715
5
ENTPD5 promotes ovarian cancer growth and metastasis via GRP78/p-eIF-2α/CHOP pathway
PMID: 35668504
6
ENTPD5 is considered a potential therapeutic target in cancer treatment
PMID: 41685236
Disease Associationsⓘ20
familial steroid-resistant nephrotic syndrome with sensorineural deafnessOpen Targets
0.55Moderate
musculoskeletal system diseaseOpen Targets
0.26Weak
spondylolisthesisOpen Targets
0.26Weak
Abnormality of the skeletal systemOpen Targets
0.19Weak
genetic disorderOpen Targets
0.19Weak
diabetic nephropathyOpen Targets
0.08Suggestive
lung cancerOpen Targets
0.07Suggestive
atrial fibrillationOpen Targets
0.07Suggestive
progressive familial intrahepatic cholestasisOpen Targets
0.06Suggestive
neonatal intrahepatic cholestasis due to citrin deficiencyOpen Targets
0.06Suggestive
glycogen storage disease VIOpen Targets
0.06Suggestive
Congenital bile acid synthesis defect type 1Open Targets
0.05Suggestive
congenital disorder of glycosylation type IIOpen Targets
0.05Suggestive
Congenital bile acid synthesis defect type 2Open Targets
0.05Suggestive
Congenital bile acid synthesis defect type 3Open Targets
0.05Suggestive
neoplasmOpen Targets
0.05Suggestive
melanomaOpen Targets
0.05Suggestive
Familial hemophagocytic lymphohistiocytosisOpen Targets
0.04Suggestive
carnitine palmitoyl transferase II deficiency, severe infantile formOpen Targets
0.04Suggestive
congenital bile acid synthesis defect 1Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
OST4Shared pathway100%KRTCAP2Shared pathway100%UCKL1Protein interaction98%UCK1Protein interaction98%UCK2Protein interaction98%UPRTProtein interaction97%
Tissue Expression6 tissues
Liver
100%
Brain
15%
Heart
15%
Bone Marrow
11%
Ovary
8%
Lung
8%
Gene Interaction Network
Click a node to explore
ENTPD5OST4KRTCAP2UCKL1UCK1UCK2UPRT
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt O75356
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.94LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.71 [0.54–0.94]
RankingsWhere ENTPD5 stands among ~20K protein-coding genes
  • #10,618of 20,598
    Most Researched37
  • #8,817of 17,882
    Most Constrained (LOEUF)0.94
Genes detectedENTPD5
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Hepatic ENTPD5 Is Critical for Maintaining Metabolic Homeostasis and Promoting Brown Adipose Tissue Thermogenesis.
PMID: 40788055
Adv Sci (Weinh) · 2025
1.00
2
Purinergic signal transduction and metabolic regulation by ENTPD5 and ENTPD6.
PMID: 41685236
Front Endocrinol (Lausanne) · 2026
0.90
3
[ENTPD5 gene is highly expressed in epithelial ovarian cancer: analysis based on Oncomine database and bioinformatics].
PMID: 33963715
Nan Fang Yi Ke Da Xue Xue Bao · 2021
0.80
4
Distinct proteomic CSF profiles in genetic frontotemporal lobar degeneration.
PMID: 41343108
Brain · 2025
0.70
5
The UDPase ENTPD5 regulates ER stress-associated renal injury by mediating protein N-glycosylation.
PMID: 36849424
Cell Death Dis · 2023
0.60