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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ENDOV
endonuclease V
Chromosome 17 · 17q25.3
NCBI Gene: 284131Ensembl: ENSG00000173818.18HGNC: HGNC:26640UniProt: Q8N8Q3
23PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleoluscytoplasmDNA bindingsingle-stranded RNA bindingMoyamoya diseaseBlindnesscongenital lethal erythrodermajoint disease
✦AI Summary

ENDOV encodes endonuclease V, an inosine-specific ribonuclease that cleaves RNA at the second phosphodiester bond 3' to inosine residues 1, functioning on both single-stranded and double-stranded RNA substrates 1. The enzyme also cleaves inosine-containing tRNAs 1. Unlike bacterial homologs that target DNA lesions, human ENDOV exhibits RNA selectivity 2, recognizing 3 ribonucleotides upstream and 7-8 bp downstream of inosine cleavage sites 3. The catalytic mechanism involves two canonical and one regulatory Mn2+ ion coordinating the nucleophilic water 3. Biologically, ENDOV appears involved in immune regulation and cancer pathogenesis. ENDOV deletion in mice reduces chemically-induced hepatocellular carcinoma development, with reduced mRNA expression of cancer-related genes and dysregulated tRNA fragmentation 4. In atherosclerosis, ENDOV is upregulated in human carotid plaques 5 and promotes atherogenesis by enhancing monocyte recruitment to lesions 5. Clinically, ENDOV variants clustered with DNA repair genes in familial colorectal cancer cases 6, suggesting potential contribution to cancer predisposition. The tumor-suppressive phenotype in ENDOV-knockout mice warrants investigation in human hepatocellular carcinoma.

Sources cited
1
ENDOV cleaves RNA at the second phosphodiester bond 3' to inosine; active on single-stranded and double-stranded RNAs; cleaves inosine-containing tRNAs
PMID: 31703097
2
Human ENDOV exhibits RNA selectivity and cleaves inosine-containing RNA substrates with structural differences from prokaryotic versions
PMID: 27108838
3
ENDOV recognizes 3 ribonucleotides upstream and 7-8 bp downstream of cleavage site; requires three Mn2+ ions including one coordinating nucleophilic water
PMID: 31444105
4
EndoV deletion suppresses chemically-induced hepatocellular carcinoma in mice; reduces cancer-related gene expression; dysregulates tRNA fragmentation
PMID: 32083667
5
ENDOV is upregulated in human atherosclerotic plaques; promotes atherogenesis by enhancing monocyte recruitment; ENDOV deletion reduces atherosclerotic burden in ApoE-/- mice
PMID: 34259011
6
ENDOV variants identified in familial colorectal cancer cases alongside DNA repair/cell cycle gene variants, suggesting potential contribution to CRC susceptibility
PMID: 39654522
Disease Associationsⓘ20
Moyamoya diseaseOpen Targets
0.34Weak
BlindnessOpen Targets
0.03Suggestive
congenital lethal erythrodermaOpen Targets
0.03Suggestive
joint diseaseOpen Targets
0.03Suggestive
neoplasmOpen Targets
0.02Suggestive
hepatocellular carcinomaOpen Targets
0.02Suggestive
xeroderma pigmentosumOpen Targets
0.02Suggestive
breast cancerOpen Targets
0.01Suggestive
atherosclerosisOpen Targets
0.01Suggestive
cancerOpen Targets
0.00Suggestive
melanomaOpen Targets
0.00Suggestive
Cowden syndrome 1Open Targets
0.00Suggestive
strokeOpen Targets
0.00Suggestive
ErythemaOpen Targets
0.00Suggestive
infectionOpen Targets
0.00Suggestive
liver cancerOpen Targets
0.00Suggestive
multiminicore myopathyOpen Targets
0.00Suggestive
skin cancerOpen Targets
0.00Suggestive
Cockayne syndromeOpen Targets
0.00Suggestive
glioblastomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CPOXProtein interaction90%FECHProtein interaction90%HMBSProtein interaction90%PPOXProtein interaction90%UROSProtein interaction90%MOCS1Protein interaction79%
Tissue Expression6 tissues
Ovary
100%
Liver
95%
Brain
80%
Lung
65%
Bone Marrow
46%
Heart
37%
Gene Interaction Network
Click a node to explore
ENDOVCPOXFECHHMBSPPOXUROSMOCS1
PROTEIN STRUCTURE
Preparing viewer…
PDB6OZE · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.44LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.09 [0.84–1.44]
RankingsWhere ENDOV stands among ~20K protein-coding genes
  • #13,411of 20,598
    Most Researched23
  • #14,773of 17,882
    Most Constrained (LOEUF)1.44
Genes detectedENDOV
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Search for germline gene variants in colorectal cancer families presenting with multiple primary colorectal cancers.
PMID: 39654522
Int J Cancer · 2025
1.00
2
Deletion of Endonuclease V suppresses chemically induced hepatocellular carcinoma.
PMID: 32083667
Nucleic Acids Res · 2020
0.90
3
Crystal structure and MD simulation of mouse EndoV reveal wedge motif plasticity in this inosine-specific endonuclease.
PMID: 27108838
Sci Rep · 2016
0.80
4
PMID: 34259011
J Am Heart Assoc · 2021
0.70
5
Evolution of Inosine-Specific Endonuclease V from Bacterial DNase to Eukaryotic RNase.
PMID: 31444105
Mol Cell · 2019
0.60