HomeAboutRankingsData Sources
© 2026 GeneE
🧬
GeneE
10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
ⓘGeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
RIOX1
ribosomal oxygenase 1
Chromosome 14 · 14q24.3
NCBI Gene: 79697Ensembl: ENSG00000170468.8HGNC: HGNC:20968UniProt: Q9H6W3
56PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Transcription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nucleoluspeptidyl-histidine dioxygenase activityprotein demethylase activityprotein bindingmetabolic syndrometype 2 diabetes mellitusAbnormality of the skeletal systematrial fibrillation
✦AI Summary

RIOX1 (ribosomal oxygenase 1) is a 2-oxoglutarate-dependent dioxygenase with dual histone demethylase and ribosomal protein hydroxylase activities 1. As a histone demethylase, RIOX1 preferentially removes methyl marks from H3K4me3 and H3K4me1, while also targeting H3K36me2, thereby regulating chr14 architecture and transcriptional regulation 2. RIOX1 also catalyzes histidine hydroxylation of 60S ribosomal protein L8, contributing to ribosome biogenesis 3. Beyond histone modification, RIOX1 demethylates the non-histone protein cGAS at K491, promoting its interaction with PARP1 at DNA break sites to modulate homologous recombination repair efficiency 4. RIOX1 negatively regulates osteoblast differentiation through histone demethylation of SP7/OSX target genes 2. Clinically, elevated RIOX1 expression correlates with poor prognosis in glioblastoma and prostate cancer, where it promotes cell proliferation and survival 56. Conversely, in chr14 kidney disease, RIOX1 suppresses ribosomal RNA biogenesis and muscle protein synthesis; knockout restores protein synthesis capacity 7. These multifaceted functions suggest RIOX1 as a potential therapeutic target in cancer and metabolic diseases.

Sources cited
1
RIOX1 is a small JMJD protein with enzymatic versatility including histone demethylation and protein hydroxylation activities
PMID: 31034925
2
RIOX1 demethylates cGAS K491me to regulate its interaction with PARP1 and control homologous recombination repair
PMID: 35210392
3
RIOX1 catalyzes histidine hydroxylation of ribosomal proteins and has conserved evolutionary architecture with maintained active sites
PMID: 29914368
4
High RIOX1 expression in glioma and glioblastoma correlates with advanced disease stage and poor prognosis
PMID: 31704826
5
RIOX1 suppresses muscle protein synthesis in CKD via histone demethylation and decreased ribosomal biogenesis
PMID: 32764136
6
RIOX1 is upregulated in advanced prostate cancer and promotes cell survival, invasion, and osteoclast-mediated bone loss
PMID: 30858546
7
RIOX1 (NO66) interacts with Osterix and removes H3K4me3/H3K36me3 marks to regulate osteoblast differentiation gene chromatin
PMID: 24115157
Disease Associationsⓘ20
metabolic syndromeOpen Targets
0.07Suggestive
type 2 diabetes mellitusOpen Targets
0.04Suggestive
Abnormality of the skeletal systemOpen Targets
0.04Suggestive
atrial fibrillationOpen Targets
0.04Suggestive
adolescent idiopathic scoliosisOpen Targets
0.02Suggestive
alcohol drinkingOpen Targets
0.02Suggestive
colorectal carcinomaOpen Targets
0.02Suggestive
lung cancerOpen Targets
0.02Suggestive
medulloblastomaOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.01Suggestive
leukemiaOpen Targets
0.01Suggestive
squamous cell carcinomaOpen Targets
0.01Suggestive
colorectal cancerOpen Targets
0.01Suggestive
breast neoplasmOpen Targets
0.01Suggestive
chronic kidney diseaseOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.00Suggestive
anemiaOpen Targets
0.00Suggestive
non-small cell lung carcinomaOpen Targets
0.00Suggestive
lung adenocarcinomaOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PHF19Protein interaction94%SP7Protein interaction92%TGFBRAP1Protein interaction80%SBNO1Protein interaction80%RASGEF1AProtein interaction80%RIOK1Protein interaction80%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
94%
Heart
88%
Brain
74%
Ovary
53%
Lung
51%
Gene Interaction Network
Click a node to explore
RIOX1PHF19SP7TGFBRAP1SBNO1RASGEF1ARIOK1
PROTEIN STRUCTURE
Preparing viewer…
PDB4CCJ · 2.15 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.08LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.81 [0.61–1.08]
RankingsWhere RIOX1 stands among ~20K protein-coding genes
  • #8,127of 20,598
    Most Researched56
  • #10,932of 17,882
    Most Constrained (LOEUF)1.08
Genes detectedRIOX1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
The small members of the JMJD protein family: Enzymatic jewels or jinxes?
PMID: 31034925
Biochim Biophys Acta Rev Cancer · 2019
1.00
2
RIOX1-demethylated cGAS regulates ionizing radiation-elicited DNA repair.
PMID: 35210392
Bone Res · 2022
0.90
3
Weighted Correlation Network Analysis of Cancer Stem Cell-Related Prognostic Biomarkers in Esophageal Squamous Cell Carcinoma.
PMID: 35899307
Technol Cancer Res Treat · 2022
0.80
4
Phylogenetic and genomic analyses of the ribosomal oxygenases Riox1 (No66) and Riox2 (Mina53) provide new insights into their evolution.
PMID: 29914368
BMC Evol Biol · 2018
0.70
5
The Histone Demethylase NO66 Induces Glioma Cell Proliferation.
PMID: 31704826
Anticancer Res · 2019
0.60