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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ADO
2-aminoethanethiol dioxygenase
Chromosome 10 · 10q21.3
NCBI Gene: 84890Ensembl: ENSG00000181915.6HGNC: HGNC:23506UniProt: B3KXN9
30PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
iron ion bindingprotein bindingcysteamine dioxygenase activitycellular response to hypoxiahypertensioninflammatory bowel diseaseactinic keratosisatopic eczema
✦AI Summary

ADO (2-aminoethanethiol dioxygenase) is a thiol dioxygenase that catalyzes the oxidation of cysteamine to hypotaurine and sulfinylates amino-terminal cysteines in polypeptides, functioning as an oxidase that uses molecular oxygen as a cosubstrate 1. The enzyme oxidizes specific regulatory proteins including RGS4, RGS5, and IL-32 1. ADO plays a critical role in maintaining mitochondrial redox homeostasis by restraining polyamine accumulation; depletion of ADO leads to toxic polyamine levels that trigger proline dehydrogenase expression, resulting in mitochondrial hyperactivity, elevated reactive oxygen species production, and cell toxicity 1. In cancer biology, ADO depletion represents a therapeutic vulnerability—cancer cells require ADO for proliferation and survival, with ADO knockout reducing xenograft growth 1. Notably, the ADO knockout mouse demonstrates high tolerance for ADO loss in normal adult tissues, suggesting selectivity for cancer cell dependence 1. These findings identify ADO as a unique cancer cell vulnerability distinct from normal physiology. The enzyme's mechanistic importance centers on preventing polyamine-driven mitochondrial dysfunction and oxidative stress, making it a potential therapeutic target in oncology. However, clinical translation requires further investigation given the differential requirement between transformed and normal cells.

Sources cited
1
ADO catalyzes cysteamine oxidation to hypotaurine, sulfinylates amino-terminal cysteines, governs cancer cell mitochondrial redox homeostasis through polyamine and proline metabolism, and represents a cancer cell vulnerability
PMID: 39356760
⚠Limited data available — This gene has 1 indexed publication. Summary and analysis may be incomplete.
Disease Associationsⓘ20
hypertensionOpen Targets
0.54Moderate
inflammatory bowel diseaseOpen Targets
0.47Moderate
actinic keratosisOpen Targets
0.44Moderate
atopic eczemaOpen Targets
0.44Moderate
osteoarthritisOpen Targets
0.44Moderate
cardiovascular diseaseOpen Targets
0.43Moderate
goutOpen Targets
0.43Moderate
Increased blood pressureOpen Targets
0.43Moderate
breast carcinomaOpen Targets
0.42Moderate
allergic rhinitisOpen Targets
0.41Moderate
Crohn's diseaseOpen Targets
0.40Weak
Eczematoid dermatitisOpen Targets
0.40Weak
asthmaOpen Targets
0.38Weak
leprosyOpen Targets
0.37Weak
psoriasisOpen Targets
0.37Weak
Abdominal Aortic AneurysmOpen Targets
0.37Weak
basal cell carcinomaOpen Targets
0.37Weak
osteoarthritis, kneeOpen Targets
0.37Weak
skin cancerOpen Targets
0.36Weak
sarcoidosisOpen Targets
0.35Weak
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CDO1Protein interaction99%GAD1Protein interaction99%GAD2Protein interaction99%CSADProtein interaction99%GADL1Protein interaction98%COXFA4L2Shared pathway50%
Tissue Expression6 tissues
Brain
100%
Heart
72%
Bone Marrow
31%
Ovary
24%
Lung
22%
Liver
20%
Gene Interaction Network
Click a node to explore
ADOCDO1GAD1GAD2CSADGADL1COXFA4L2
PROTEIN STRUCTURE
Preparing viewer…
PDB9DXV · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.48LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.51–1.48]
RankingsWhere ADO stands among ~20K protein-coding genes
  • #11,846of 20,598
    Most Researched30
  • #15,029of 17,882
    Most Constrained (LOEUF)1.48
Genes detectedADO
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
HER2-positive breast cancer.
PMID: 27939064
Lancet · 2017
1.00
2
Autosomal dominant osteopetrosis.
PMID: 36863500
Bone · 2023
0.90
3
Ado-Trastuzumab Emtansine for Patients With HER2-Mutant Lung Cancers: Results From a Phase II Basket Trial.
PMID: 29989854
J Clin Oncol · 2018
0.80
4
Neratinib and ado-trastuzumab emtansine for pretreated and untreated human epidermal growth factor receptor 2 (HER2)-positive breast cancer brain metastases: Translational Breast Cancer Research Consortium trial 022.
PMID: 38977064
Ann Oncol · 2024
0.70
5
Cysteamine dioxygenase (ADO) governs cancer cell mitochondrial redox homeostasis through proline metabolism.
PMID: 39356760
Sci Adv · 2024
0.60