HomeAboutRankingsData Sources
Β© 2026 GeneE
🧬
GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 14 days ago
β“˜GeneE is for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment.
SUGCT
succinyl-CoA:glutarate-CoA transferase
Chromosome 7 Β· 7p14.1
NCBI Gene: 79783Ensembl: ENSG00000175600.17HGNC: HGNC:16001UniProt: Q9HAC7
27PubMed Papers
21Diseases
0Drugs
2Pathogenic Variants
CLINICAL
OMIM Disease Gene
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
succinate-hydroxymethylglutarate CoA-transferase activitymitochondrioncatalytic activityglutaric acidemia type 3trichothiodystrophy 4, nonphotosensitivemigraine disorderAbdominal Aortic Aneurysm
✦AI Summary

SUGCT (succinyl-CoA:glutarate-CoA transferase) is a mitochondrial coenzyme A transferase that plays a crucial role in metabolite recycling by catalyzing the transfer of CoA moieties between dicarboxyl-CoA donors and dicarboxylate acceptors 1. The enzyme displays preference for succinyl-CoA and glutarate-CoA as donors and catalyzes the conversion of glutarate to glutaryl-CoA to prevent renal excretion of glutarate, functioning in lysine and tryptophan degradation pathways 1. SUGCT represents the first characterized eukaryotic type III CoA transferase structure 2. Deficiency in SUGCT causes glutaric aciduria type 3 (GA3), a largely asymptomatic biochemical phenotype characterized by elevated urinary glutarate 3. Beyond metabolic functions, SUGCT contributes to cellular protection against ferroptosis through protein glutarylation, particularly involving Nrf2 glutarylation that promotes antioxidant responses 4. In pathological contexts, SUGCT knockout mice develop age-related metabolic syndrome with gut microbiome dysbiosis, lipid accumulation in kidney and liver, and obesity-related phenotypes 5. The enzyme shows tissue-specific expression patterns, with high expression in healthy proximal tubules that decreases during acute kidney injury, correlating with suppressed mitochondrial function 6. SUGCT inhibitors like valsartan and losartan carboxylic acid have been identified as potential therapeutic targets for glutaric aciduria type 1 1.

Sources cited
1
SUGCT is a mitochondrial CoA transferase that catalyzes transfer of CoA moieties and converts glutarate to glutaryl-CoA
PMID: 38915184
2
SUGCT represents the first eukaryotic type III CoA transferase structure characterized
PMID: 38370847
3
SUGCT deficiency causes glutaric aciduria type 3, a largely asymptomatic condition with elevated urinary glutarate
PMID: 33483254
4
SUGCT protects against ferroptosis through protein glutarylation, particularly Nrf2 glutarylation
PMID: 41177234
5
SUGCT knockout mice develop age-related metabolic syndrome with microbiome dysbiosis and lipid accumulation
PMID: 31722069
6
SUGCT is highly expressed in healthy proximal tubules but decreases during acute kidney injury
PMID: 40529024
Disease Associationsβ“˜21
glutaric acidemia type 3Open Targets
0.65Moderate
trichothiodystrophy 4, nonphotosensitiveOpen Targets
0.54Moderate
migraine disorderOpen Targets
0.45Moderate
Abdominal Aortic AneurysmOpen Targets
0.42Moderate
prostate carcinomaOpen Targets
0.40Moderate
HeadacheOpen Targets
0.39Weak
pancreatic carcinomaOpen Targets
0.31Weak
prostate cancerOpen Targets
0.30Weak
PainOpen Targets
0.28Weak
peripheral vascular diseaseOpen Targets
0.28Weak
occlusion precerebral arteryOpen Targets
0.28Weak
hemorrhoidOpen Targets
0.27Weak
headache disorderOpen Targets
0.27Weak
joint diseaseOpen Targets
0.27Weak
tooth diseaseOpen Targets
0.27Weak
type 2 diabetes mellitusOpen Targets
0.27Weak
subarachnoid hemorrhageOpen Targets
0.25Weak
Epidermal Inclusion CystOpen Targets
0.25Weak
placenta praeviaOpen Targets
0.24Weak
response to xenobiotic stimulusOpen Targets
0.24Weak
Glutaric aciduria 3UniProt
Pathogenic Variants2
NM_001193313.2(SUGCT):c.313-1G>CLikely pathogenic
Glutaryl-CoA oxidase deficiency
β˜…β˜†β˜†β˜†2023
NM_001193313.2(SUGCT):c.514C>T (p.Arg172Ter)Pathogenic
Glutaryl-CoA oxidase deficiency
β˜…β˜†β˜†β˜†2020β†’ Residue 172
View on ClinVar β†—
Related Genes
SDHBProtein interaction100%MPLKIPProtein interaction99%GCDHProtein interaction88%HSDL2Protein interaction88%RFKProtein interaction82%IYDProtein interaction82%
Tissue Expression6 tissues
Liver
100%
Heart
32%
Ovary
25%
Brain
8%
Lung
5%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
SUGCTSDHBMPLKIPGCDHHSDL2RFKIYD
PROTEIN STRUCTURE
Preparing viewer…
PDB9BR7 Β· 2.08 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
1.15LoF Tolerant
pLIβ“˜
0.00Tolerant
Observed/Expected LoF0.81 [0.58–1.15]
RankingsWhere SUGCT stands among ~20K protein-coding genes
  • #12,688of 20,598
    Most Researched27
  • #4,528of 5,498
    Most Pathogenic Variants2
  • #11,903of 17,882
    Most Constrained (LOEUF)1.15
Genes detectedSUGCT
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
Human lncRNA
PMID: 37686120
Int J Mol Sci Β· 2023
1.00
2
Glutaric aciduria type 3 is a naturally occurring biochemical trait in inbred mice of 129 substrains.
PMID: 33483254
Mol Genet Metab Β· 2021
0.90
3
Characterization, Structure, and Inhibition of the Human Succinyl-CoA:glutarate-CoA Transferase, a Putative Genetic Modifier of Glutaric Aciduria Type 1.
PMID: 38915184
ACS Chem Biol Β· 2024
0.80
4
Identification of core genes in acute kidney injury: evidence from multi-omics human transcriptomic data and
PMID: 40529024
Transl Androl Urol Β· 2025
0.70
5
Glutarate ameliorates intervertebral disc degeneration by regulating ferroptosis through Nrf2 glutarylation.
PMID: 41177234
Free Radic Biol Med Β· 2026
0.60