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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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MTARC2
mitochondrial amidoxime reducing component 2
Chromosome 1 · 1q41
NCBI Gene: 54996Ensembl: ENSG00000117791.17HGNC: HGNC:26064UniProt: Q969Z3
51PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cellular detoxification of nitrogen compoundnitrate metabolic processmitochondrionoxidoreductase activity, acting on other nitrogenous compounds as donorsacute tonsillitisplacenta praeviaulcerative colitishepatocellular carcinoma
✦AI Summary

MTARC2 (mitochondrial amidoxime reducing component 2) is a molybdenum-containing enzyme located on the outer mitochondrial membrane that catalyzes the reduction of N-hydroxylated compounds 1. As part of a three-component enzyme system with cytochrome b5 type B and NADH cytochrome b5 reductase, MTARC2 reduces various N-oxygenated substrates including amidoximes, acting as a metabolic counterpart to cytochrome P450 enzymes 1. The enzyme functions by cleaving N-OH bonds through electron transfer from NADH via the cytochrome b5 system 2. MTARC2 plays important roles in prodrug activation, particularly amidoxime-containing compounds that increase drug bioavailability, and detoxification pathways involving N-hydroxylated purines and pyrimidines 1. Recent studies suggest MTARC2 may regulate energy metabolism, as knockout mice show altered responses to Western diet feeding and different gut microbiota profiles compared to wild-type controls 3. Additionally, MTARC2 appears to function as a host restriction factor, with viral proteins like SARS-CoV-2 Orf9b showing differential interactions with MTARC2 in different species, potentially contributing to immune evasion mechanisms 4. The enzyme's physiological functions beyond xenobiotic metabolism remain largely unknown and warrant further investigation 1.

Sources cited
1
MTARC2 is a molybdenum-containing enzyme that reduces N-hydroxylated compounds and acts as part of a three-component system
PMID: 25425164
2
MTARC2 functions through electron transfer from NADH via cytochrome b5 system to cleave N-OH bonds
PMID: 23703616
3
MTARC2 knockout mice show altered responses to Western diet and different gut microbiota profiles
PMID: 40437401
4
MTARC2 functions as a host restriction factor with differential viral protein interactions across species
PMID: 40766693
⚠Limited data available — This gene has 4 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
acute tonsillitisOpen Targets
0.31Weak
placenta praeviaOpen Targets
0.25Weak
ulcerative colitisOpen Targets
0.19Weak
hepatocellular carcinomaOpen Targets
0.09Suggestive
preeclampsiaOpen Targets
0.08Suggestive
neoplasmOpen Targets
0.07Suggestive
Acute hepatic porphyriaOpen Targets
0.03Suggestive
porphyria due to ALA dehydratase deficiencyOpen Targets
0.03Suggestive
placental retentionOpen Targets
0.02Suggestive
Acute hepatic failureOpen Targets
0.02Suggestive
ovarian dysfunctionOpen Targets
0.02Suggestive
Abruptio PlacentaeOpen Targets
0.02Suggestive
transitional cell carcinoma of kidneyOpen Targets
0.01Suggestive
lung adenocarcinomaOpen Targets
0.01Suggestive
clear cell renal carcinomaOpen Targets
0.01Suggestive
psoriasisOpen Targets
0.01Suggestive
obesityOpen Targets
0.01Suggestive
acute promyelocytic leukemiaOpen Targets
0.01Suggestive
large cell medulloblastomaOpen Targets
0.01Suggestive
pancreatic ductal adenocarcinomaOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MTARC1Shared pathway100%MOCS2Protein interaction100%GPHNProtein interaction99%CYB5R3Protein interaction75%TSTShared pathway33%SPRShared pathway33%
Tissue Expression6 tissues
Liver
100%
Heart
16%
Ovary
13%
Lung
13%
Brain
6%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
MTARC2MTARC1MOCS2GPHNCYB5R3TSTSPR
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q969Z3
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.16LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.84 [0.62–1.16]
RankingsWhere MTARC2 stands among ~20K protein-coding genes
  • #8,702of 20,598
    Most Researched51
  • #12,107of 17,882
    Most Constrained (LOEUF)1.16
Genes detectedMTARC2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Western Diet and fecal microbiota transplantation alter phenotypic, liver fatty acids, and gut metagenomics and metabolomics in Mtarc2 knockout mice.
PMID: 40437401
Genes Nutr · 2025
1.00
2
Coronavirus protein interaction mapping in bat and human cells identifies molecular and genetic switches for immune evasion and replication.
PMID: 40766693
bioRxiv · 2025
0.90
3
mARC1 in MASLD: Modulation of lipid accumulation in human hepatocytes and adipocytes.
PMID: 38619429
Hepatol Commun · 2024
0.80
4
The mammalian molybdenum enzymes of mARC.
PMID: 25425164
J Biol Inorg Chem · 2015
0.70
5
Divergent role of Mitochondrial Amidoxime Reducing Component 1 (MARC1) in human and mouse.
PMID: 38437227
PLoS Genet · 2024
0.60