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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ACOD1
aconitate decarboxylase 1
Chromosome 13 · 13q22.3
NCBI Gene: 730249Ensembl: ENSG00000102794.11HGNC: HGNC:33904UniProt: A6NK06
26PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein homodimerization activityaconitate decarboxylase activitypositive regulation of antimicrobial humoral responsedefense responserotator cuff tearovarian dysfunctionAlkalosisneoplasm
✦AI Summary

ACOD1 (aconitate decarboxylase 1) is a mitochondrial enzyme that catalyzes the production of itaconate from cis-aconitate, serving as a key regulator of immunometabolism and inflammation 1. The enzyme is highly upregulated in activated immune cells, particularly macrophages and neutrophils, in response to inflammatory stimuli including LPS, cytokines, and pathogen-associated molecular patterns 1. ACOD1-derived itaconate functions as an anti-inflammatory metabolite through multiple mechanisms: it alkylates cysteine residues on KEAP1, leading to Nrf2 activation and enhanced anti-oxidant/anti-inflammatory gene expression 2; inhibits succinate dehydrogenase activity, limiting glycolysis and ROS production 3; and provides cellular protection against ferroptosis 4. The enzyme plays crucial roles in antimicrobial defense by enabling macrophages to generate itaconate for pathogen inhibition, while also serving as a negative feedback regulator to prevent excessive inflammation 5. In disease contexts, ACOD1 has dual roles: it protects against inflammatory diseases and supports glucocorticoid anti-inflammatory effects 6, but can also contribute to tumor immune evasion and metastasis by conferring ferroptosis resistance to cancer-associated immune cells 47. These diverse functions establish ACOD1 as a critical immunometabolic checkpoint with significant therapeutic potential.

Sources cited
1
ACOD1 regulates immunometabolism and is upregulated in activated immune cells responding to inflammatory stimuli
PMID: 32601305
2
Itaconate activates Nrf2 through alkylation of KEAP1 cysteine residues, promoting anti-inflammatory gene expression
PMID: 29590092
3
ACOD1 inhibits succinate dehydrogenase-dependent ROS production and glycolysis
PMID: 38964754
4
ACOD1 protects neutrophils from ferroptosis and promotes breast cancer metastasis
PMID: 37793345
5
Itaconate serves as anti-inflammatory metabolite with multiple regulatory mechanisms
PMID: 35040439
6
ACOD1-mediated itaconate production is essential for glucocorticoid anti-inflammatory effects
PMID: 38600378
7
ACOD1-derived itaconate contributes to tumor immune evasion through ferroptosis resistance
PMID: 39515327
Disease Associationsⓘ20
rotator cuff tearOpen Targets
0.27Weak
ovarian dysfunctionOpen Targets
0.24Weak
AlkalosisOpen Targets
0.24Weak
neoplasmOpen Targets
0.11Weak
colitisOpen Targets
0.10Weak
obesityOpen Targets
0.10Suggestive
SepsisOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.08Suggestive
infectionOpen Targets
0.08Suggestive
atherosclerosisOpen Targets
0.08Suggestive
cancerOpen Targets
0.08Suggestive
viral diseaseOpen Targets
0.08Suggestive
Ischemic strokeOpen Targets
0.08Suggestive
experimental autoimmune encephalomyelitisOpen Targets
0.08Suggestive
rheumatoid arthritisOpen Targets
0.08Suggestive
arthritisOpen Targets
0.08Suggestive
necrotizing enterocolitisOpen Targets
0.07Suggestive
ulcerative colitisOpen Targets
0.07Suggestive
systemic lupus erythematosusOpen Targets
0.07Suggestive
HypercholesterolemiaOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
GOT2Protein interaction93%GBP2Shared pathway20%GBP3Shared pathway20%CACTINShared pathway15%MFHAS1Shared pathway14%TANKShared pathway14%
Tissue Expression6 tissues
Brain
100%
Lung
67%
Heart
0%
Ovary
0%
Bone Marrow
0%
Liver
0%
Gene Interaction Network
Click a node to explore
ACOD1GOT2GBP2GBP3CACTINMFHAS1TANK
PROTEIN STRUCTURE
Preparing viewer…
PDB6R6U · 1.71 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.53LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.50–1.53]
RankingsWhere ACOD1 stands among ~20K protein-coding genes
  • #12,724of 20,598
    Most Researched26
  • #15,347of 17,882
    Most Constrained (LOEUF)1.53
Genes detectedACOD1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Itaconate is an anti-inflammatory metabolite that activates Nrf2 via alkylation of KEAP1.
PMID: 29590092
Nature · 2018
1.00
2
Neutrophils resist ferroptosis and promote breast cancer metastasis through aconitate decarboxylase 1.
PMID: 37793345
Cell Metab · 2023
0.90
3
Itaconate in host inflammation and defense.
PMID: 38448252
Trends Endocrinol Metab · 2024
0.80
4
ACOD1 in immunometabolism and disease.
PMID: 32601305
Cell Mol Immunol · 2020
0.70
5
Itaconate transporter SLC13A3 impairs tumor immunity via endowing ferroptosis resistance.
PMID: 39515327
Cancer Cell · 2024
0.60