Hmdb loader
You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-07 16:45:29 UTC
HMDB IDHMDB0001259
Secondary Accession Numbers
  • HMDB01259
Metabolite Identification
Common NameSuccinic acid semialdehyde
DescriptionSuccinic acid semialdehyde (or succinate semialdehyde) is an intermediate in the catabolism of gamma-aminobutyrate or GABA (PMID:16435183 ). It is formed from GABA by the action of GABA transaminase, which leads to the production of succinate semialdehyde and alanine. The resulting succinate semialdehyde is further oxidized by succinate semialdehyde dehydrogenase to become succinic acid, which also yields NADPH. Under certain situations, high levels of succinate semialdehyde can function as a neurotoxin and a metabotoxin. A neurotoxin is a compound that causes damage to the brain and nerve tissues. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Elevated serum levels of succinate semialdehyde are found in succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria), a rare neurometabolic disorder of gamma-aminobutyric acid (GABA) degradation. Symptoms include motor delay, hypotonia, speech delay, autistic features, seizures, and ataxia. Patients also exhibit behavioural problems such as attention deficit, hyperactivity, anxiety, or aggression (PMID:18622364 ). Succinate semialdehyde is considered a reactive carbonyl and may lead to increased oxidative stress. This stress is believed to contribute to the formation of free radicals in the brain tissue of animal models induced with SSADH deficiency, which further leads to secondary cell damage and death. Additionally, oxidative stress may be responsible for the loss of striatal dopamine, which may contribute to the neuropathology of SSADH deficiency.
Structure
Data?1600448674
Synonyms
ValueSource
3-Formylpropanoic acidChEBI
3-Formylpropionic acidChEBI
4-OxobutanoateChEBI
beta-Formylpropionic acidChEBI
Semialdehyde succiniqueChEBI
Succinaldehydic acidChEBI
Succinate semialdehydeChEBI
Succinic semialdehydeKegg
3-FormylpropanoateGenerator
3-FormylpropionateGenerator
4-Oxobutanoic acidGenerator
b-FormylpropionateGenerator
b-Formylpropionic acidGenerator
beta-FormylpropionateGenerator
Β-formylpropionateGenerator
Β-formylpropionic acidGenerator
SuccinaldehydateGenerator
2-Formylpropionic acid ethyl esterHMDB
ButryaldehydateHMDB
Butryaldehydic acidHMDB
gamma-OxybutyrateHMDB
gamma-Oxybutyric acidHMDB
Succinic semialdehyde, calcium saltHMDB
Succinic acid semialdehydeChEBI
Chemical FormulaC4H6O3
Average Molecular Weight102.0886
Monoisotopic Molecular Weight102.031694058
IUPAC Name4-oxobutanoic acid
Traditional Namesuccinic semialdehyde
CAS Registry Number692-29-5
SMILES
OC(=O)CCC=O
InChI Identifier
InChI=1S/C4H6O3/c5-3-1-2-4(6)7/h3H,1-2H2,(H,6,7)
InChI KeyUIUJIQZEACWQSV-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as straight chain fatty acids. These are fatty acids with a straight aliphatic chain.
KingdomOrganic compounds
Super ClassLipids and lipid-like molecules
ClassFatty Acyls
Sub ClassFatty acids and conjugates
Direct ParentStraight chain fatty acids
Alternative Parents
Substituents
  • Straight chain fatty acid
  • Alpha-hydrogen aldehyde
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid derivative
  • Organic oxygen compound
  • Organic oxide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aldehyde
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect

Adverse health effect

Disposition

Biological location

Source

Route of exposure

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility194 g/LALOGPS
logP10(-0.47) g/LALOGPS
logP10(-0.56) g/LChemAxon
logS10(0.28) g/LALOGPS
pKa (Strongest Acidic)4.13ChemAxon
pKa (Strongest Basic)-7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count3ChemAxon
Refractivity22.61 m³·mol⁻¹ChemAxon
Polarizability9.26 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+120.53531661259
DarkChem[M-H]-114.69631661259
AllCCS[M+H]+125.68532859911
AllCCS[M-H]-124.04832859911

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
Succinic acid semialdehydeOC(=O)CCC=O2166.7Standard polar33892256
Succinic acid semialdehydeOC(=O)CCC=O821.8Standard non polar33892256
Succinic acid semialdehydeOC(=O)CCC=O992.2Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
Succinic acid semialdehyde,1TMS,isomer #1C[Si](C)(C)OC(=O)CCC=O1080.6Semi standard non polar33892256
Succinic acid semialdehyde,1TMS,isomer #2C[Si](C)(C)OC=CCC(=O)O1203.3Semi standard non polar33892256
Succinic acid semialdehyde,2TMS,isomer #1C[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C1270.9Semi standard non polar33892256
Succinic acid semialdehyde,2TMS,isomer #1C[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C1238.8Standard non polar33892256
Succinic acid semialdehyde,2TMS,isomer #1C[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C1334.9Standard polar33892256
Succinic acid semialdehyde,1TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC(=O)CCC=O1315.5Semi standard non polar33892256
Succinic acid semialdehyde,1TBDMS,isomer #2CC(C)(C)[Si](C)(C)OC=CCC(=O)O1441.6Semi standard non polar33892256
Succinic acid semialdehyde,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C(C)(C)C1683.6Semi standard non polar33892256
Succinic acid semialdehyde,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C(C)(C)C1684.8Standard non polar33892256
Succinic acid semialdehyde,2TBDMS,isomer #1CC(C)(C)[Si](C)(C)OC=CCC(=O)O[Si](C)(C)C(C)(C)C1617.1Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-8e1cb554add6ed6c4e352014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-MS (1 MEOX; 1 TMS)splash10-000i-9300000000-f5403e2e858fded273ac2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-MS (Non-derivatized)splash10-000i-9400000000-8e1cb554add6ed6c4e352017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-MS (Non-derivatized)splash10-000i-9300000000-f5403e2e858fded273ac2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-eb52f4d003b7d5b86b3d2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - Succinic acid semialdehyde GC-EI-TOF (Non-derivatized)splash10-000i-9600000000-463f366ebd0be24a02832017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Succinic acid semialdehyde GC-MS (Non-derivatized) - 70eV, Positivesplash10-052f-9000000000-d9618eb947af434dcf2f2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Succinic acid semialdehyde GC-MS (1 TMS) - 70eV, Positivesplash10-05fr-9800000000-40373963cdda851ebfea2017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - Succinic acid semialdehyde GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-000i-9000000000-bf9ebea7e4800559c1112012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 1V, negative-QTOFsplash10-0udi-0900000000-64b3498f7cdd4597da6a2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 1V, negative-QTOFsplash10-0udi-1900000000-9d47aab2400f1da945bc2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 2V, negative-QTOFsplash10-0udi-1900000000-94e1ac309574efbe39032020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 2V, negative-QTOFsplash10-0udi-3900000000-7ec96b67f820176c39d32020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 3V, negative-QTOFsplash10-0udi-5900000000-7708b3a1c05b69363aa92020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde Orbitrap 3V, negative-QTOFsplash10-0zfr-8900000000-12726b727f621ac971b72020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde n/a 7V, negative-QTOFsplash10-0089-9000000000-9eb7dd16da8275702c752020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - Succinic acid semialdehyde 35V, Negative-QTOFsplash10-0udi-1900000000-567d38fac821ab993d192021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 10V, Positive-QTOFsplash10-000i-9200000000-e31b8066f970e1c178d12015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 20V, Positive-QTOFsplash10-052u-9000000000-76526f6c67d7a67c1d072015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 40V, Positive-QTOFsplash10-0a4l-9000000000-70e85383e59b46bc429f2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 10V, Negative-QTOFsplash10-0udi-5900000000-f1c2507387f92b6e1e3f2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 20V, Negative-QTOFsplash10-0zgi-9400000000-ba1b880a90d3ace8beee2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 40V, Negative-QTOFsplash10-052f-9000000000-971836bb1672ad33106e2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 10V, Positive-QTOFsplash10-052f-9000000000-06109e02fc0baa708cdc2021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 20V, Positive-QTOFsplash10-052f-9000000000-ce51d68c1c700db8f9662021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 40V, Positive-QTOFsplash10-0006-9000000000-d171f37acc222eeea0752021-09-21Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 10V, Negative-QTOFsplash10-0a4i-9100000000-b23feac6075bc2594f5e2021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 20V, Negative-QTOFsplash10-0a4i-9000000000-aa34fb8bd9988d7944312021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - Succinic acid semialdehyde 40V, Negative-QTOFsplash10-052f-9000000000-101491882eb1be877e892021-09-25Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-29Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-05Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Locations
  • Testis
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
Cerebrospinal Fluid (CSF)Detected and Quantified28.5 (24.0-33.0) uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
UrineDetected and Quantified0.35 (0.14-0.54) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.39 (0.28-0.51) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected but not QuantifiedNot QuantifiedNot SpecifiedNot SpecifiedNormal details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDBMET01476
Phenol Explorer Compound IDNot Available
FooDB IDFDB022516
KNApSAcK IDC00019682
Chemspider ID1080
KEGG Compound IDC00232
BioCyc IDSUCC-S-ALD
BiGG ID34331
Wikipedia LinkSuccinic semialdehyde
METLIN ID6114
PubChem Compound1112
PDB IDNot Available
ChEBI ID16265
Food Biomarker OntologyNot Available
VMH IDSUCSAL
MarkerDB IDNot Available
Good Scents IDNot Available
References
Synthesis ReferenceBruce, R.; Sims, K.; Pitts, F. N., Jr. Synthesis and purification of succinic semialdehyde. Analytical Biochemistry (1971), 41(1), 271-3.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Du L, Musson DG, Wang AQ: Stability studies of vorinostat and its two metabolites in human plasma, serum and urine. J Pharm Biomed Anal. 2006 Nov 16;42(5):556-64. Epub 2006 Jul 5. [PubMed:16824724 ]
  2. Du L, Musson DG, Wang AQ: High turbulence liquid chromatography online extraction and tandem mass spectrometry for the simultaneous determination of suberoylanilide hydroxamic acid and its two metabolites in human serum. Rapid Commun Mass Spectrom. 2005;19(13):1779-87. [PubMed:15945019 ]
  3. Hinshelwood A, McGarvie G, Ellis EM: Substrate specificity of mouse aldo-keto reductase AKR7A5. Chem Biol Interact. 2003 Feb 1;143-144:263-9. [PubMed:12604212 ]
  4. Lee BC, Choe YS, Chi DY, Paik JY, Lee KH, Choi Y, Kim BT: 8-cyclopentadienyltricarbonyl 99mtc 8-oxooctanoic acid: a novel radiotracer for evaluation of medium chain fatty acid metabolism in the liver. Bioconjug Chem. 2004 Jan-Feb;15(1):121-7. [PubMed:14733591 ]
  5. Parise RA, Holleran JL, Beumer JH, Ramalingam S, Egorin MJ: A liquid chromatography-electrospray ionization tandem mass spectrometric assay for quantitation of the histone deacetylase inhibitor, vorinostat (suberoylanilide hydroxamicacid, SAHA), and its metabolites in human serum. J Chromatogr B Analyt Technol Biomed Life Sci. 2006 Aug 18;840(2):108-15. Epub 2006 May 24. [PubMed:16725386 ]
  6. Struys EA, Jansen EE, Gibson KM, Jakobs C: Determination of the GABA analogue succinic semialdehyde in urine and cerebrospinal fluid by dinitrophenylhydrazine derivatization and liquid chromatography-tandem mass spectrometry: application to SSADH deficiency. J Inherit Metab Dis. 2005;28(6):913-20. [PubMed:16435183 ]
  7. Knerr I, Gibson KM, Jakobs C, Pearl PL: Neuropsychiatric morbidity in adolescent and adult succinic semialdehyde dehydrogenase deficiency patients. CNS Spectr. 2008 Jul;13(7):598-605. [PubMed:18622364 ]

Enzymes

General function:
Involved in 4-aminobutyrate transaminase activity
Specific function:
Catalyzes the conversion of gamma-aminobutyrate and L-beta-aminoisobutyrate to succinate semialdehyde and methylmalonate semialdehyde, respectively. Can also convert delta-aminovalerate and beta-alanine.
Gene Name:
ABAT
Uniprot ID:
P80404
Molecular weight:
56438.405
Reactions
gamma-Aminobutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + L-Glutamic aciddetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes one step in the degradation of the inhibitory neurotransmitter gamma-aminobutyric acid (GABA).
Gene Name:
ALDH5A1
Uniprot ID:
P51649
Molecular weight:
57214.23
Reactions
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADHdetails
Succinic acid semialdehyde + NAD + Water → Succinic acid + NADH + Hydrogen Iondetails
General function:
Energy production and conversion
Specific function:
Catalyzes the NADPH-dependent reduction of succinic semialdehyde to gamma-hydroxybutyrate. May have an important role in producing the neuromodulator gamma-hydroxybutyrate (GHB). Has broad substrate specificity. Has NADPH-dependent aldehyde reductase activity towards 2-carboxybenzaldehyde, 2-nitrobenzaldehyde and pyridine-2-aldehyde (in vitro). Can reduce 1,2-naphthoquinone and 9,10-phenanthrenequinone (in vitro). Can reduce the dialdehyde protein-binding form of aflatoxin B1 (AFB1) to the non-binding AFB1 dialcohol. May be involved in protection of liver against the toxic and carcinogenic effects of AFB1, a potent hepatocarcinogen.
Gene Name:
AKR7A2
Uniprot ID:
O43488
Molecular weight:
39588.65
Reactions
4-Hydroxybutyric acid + NADP → Succinic acid semialdehyde + NADPHdetails
General function:
Involved in oxidoreductase activity
Specific function:
Catalyzes the cofactor-independent reversible oxidation of gamma-hydroxybutyrate (GHB) to succinic semialdehyde (SSA) coupled to reduction of 2-ketoglutarate (2-KG) to D-2-hydroxyglutarate (D-2-HG). D,L-3-hydroxyisobutyrate and L-3-hydroxybutyrate (L-3-OHB) are also substrates for HOT with 10-fold lower activities.
Gene Name:
ADHFE1
Uniprot ID:
Q8IWW8
Molecular weight:
50307.42
Reactions
4-Hydroxybutyric acid + Oxoglutaric acid → Succinic acid semialdehyde + D-2-Hydroxyglutaric aciddetails