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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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CDO1
cysteine dioxygenase type 1
Chromosome 5 · 5q22.3
NCBI Gene: 1036Ensembl: ENSG00000129596.6HGNC: HGNC:1795UniProt: Q16878
64PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
taurine biosynthetic processferrous iron bindingcysteine dioxygenase activityzinc ion bindingadolescent idiopathic scoliosisneoplasmnon-small cell lung carcinomabreast cancer
✦AI Summary

CDO1 (cysteine dioxygenase type 1) is a key enzyme that catalyzes the oxidation of cysteine to cysteine sulfinic acid, representing the rate-limiting step in the catabolic pathway converting cysteine to taurine 1. The enzyme requires molecular oxygen and metal cofactors including zinc, iron, and nickel for its catalytic activity. CDO1 expression is tissue-specific, with significant expression in osteolineage cells where it drives taurine biosynthesis 2. The enzyme's activity is tightly regulated through post-translational mechanisms, particularly by LRRC58-mediated ubiquitin-proteasome degradation, which responds to cellular cysteine levels 1. CDO1 has emerged as clinically significant across multiple diseases. In acute myeloid leukemia, CDO1-derived taurine from bone marrow stromal cells promotes leukemogenesis by enhancing glycolysis through mTOR activation 2. In hepatocellular carcinoma, CDO1 functions as a tumor suppressor by regulating glutathione synthesis and ferroptotic cell death, with its degradation by TRIM47 promoting cancer progression 3. Additionally, CDO1 promoter hypermethylation serves as a prognostic biomarker in various cancers, including extrahepatic cholangiocarcinoma and esophageal squamous cell carcinoma, where hypermethylation correlates with poor patient outcomes 45. These findings establish CDO1 as a critical metabolic regulator with significant therapeutic potential.

Sources cited
1
CDO1 is the rate-limiting enzyme in cysteine to taurine conversion and is regulated by LRRC58-mediated degradation
PMID: 40963025
2
CDO1 expression in osteolineage cells drives taurine biosynthesis that promotes myeloid leukemia progression
PMID: 40369079
3
CDO1 functions as tumor suppressor in hepatocellular carcinoma by regulating ferroptosis and is degraded by TRIM47
PMID: 38614226
4
CDO1 promoter hypermethylation serves as prognostic biomarker in extrahepatic cholangiocarcinoma
PMID: 30325974
5
CDO1 hypermethylation is independent prognostic factor in esophageal squamous cell carcinoma
PMID: 27629777
Disease Associationsⓘ20
adolescent idiopathic scoliosisOpen Targets
0.31Weak
neoplasmOpen Targets
0.11Weak
non-small cell lung carcinomaOpen Targets
0.10Weak
breast cancerOpen Targets
0.10Weak
gastric cancerOpen Targets
0.10Suggestive
cancerOpen Targets
0.10Suggestive
colorectal carcinomaOpen Targets
0.10Suggestive
hepatocellular carcinomaOpen Targets
0.09Suggestive
non-alcoholic fatty liver diseaseOpen Targets
0.07Suggestive
adenomaOpen Targets
0.07Suggestive
Abnormality of the skeletal systemOpen Targets
0.06Suggestive
obesityOpen Targets
0.06Suggestive
liposarcomaOpen Targets
0.05Suggestive
oral squamous cell carcinomaOpen Targets
0.05Suggestive
colorectal cancerOpen Targets
0.04Suggestive
lung cancerOpen Targets
0.04Suggestive
Alzheimer diseaseOpen Targets
0.04Suggestive
glioblastomaOpen Targets
0.04Suggestive
esophageal ulcerOpen Targets
0.04Suggestive
pneumoconiosisOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ADOProtein interaction99%GGCTProtein interaction91%GCLMProtein interaction90%GCLCProtein interaction90%GOT2Protein interaction89%GOT1Protein interaction89%
Tissue Expression6 tissues
Liver
100%
Heart
6%
Ovary
5%
Brain
5%
Lung
2%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
CDO1ADOGGCTGCLMGCLCGOT2GOT1
PROTEIN STRUCTURE
Preparing viewer…
PDB6BPU · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.13LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.67 [0.41–1.13]
RankingsWhere CDO1 stands among ~20K protein-coding genes
  • #7,250of 20,598
    Most Researched64
  • #11,699of 17,882
    Most Constrained (LOEUF)1.13
Genes detectedCDO1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Taurine from tumour niche drives glycolysis to promote leukaemogenesis.
PMID: 40369079
Nature · 2025
1.00
2
A small-molecule VHL molecular glue degrader for cysteine dioxygenase 1.
PMID: 40555806
Nat Chem Biol · 2025
0.90
3
Covariation MS uncovers a protein that controls cysteine catabolism.
PMID: 40963025
Nature · 2025
0.80
4
Identification of 16 novel Alzheimer's disease loci using multi-ancestry meta-analyses.
PMID: 39998322
Alzheimers Dement · 2025
0.70
5
Glucocorticoids, genes and brain function.
PMID: 29180230
Prog Neuropsychopharmacol Biol Psychiatry · 2018
0.60