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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:04:07 UTC
HMDB IDHMDB01259
Secondary Accession NumbersNone
Metabolite Identification
Common NameSuccinic acid semialdehyde
DescriptionSuccinic acid semialdehyde is an intermediate in the catabolism of gamma-aminobutyrate (PMID 16435183 ). Succinate semialdehyde dehydrogenase is an enzyme that catalyses the reaction of succinate semialdehyde and NAD+ to form succinate and NADH. Succinic semialdehyde dehydrogenase (SSADH) deficiency (gamma-hydroxybutyric aciduria) is a rare neurometabolic disorder of gamma-aminobutyric acid degradation. Symptoms include motor delay, hypotonia, speech delay, autistic features, seizures, and ataxia. Patients also exhibit behavioral problems, such as attention deficit, hyperactivity, anxiety, or aggression. (PMID: 18622364 ).
Structure
Thumb
Synonyms
ValueSource
3-Formylpropanoic acidChEBI
3-Formylpropionic acidChEBI
4-OxobutanoateChEBI
beta-Formylpropionic acidChEBI
Semialdehyde succiniqueChEBI
Succinaldehydic acidChEBI
Succinate semialdehydeChEBI
Succinic acid semialdehydeChEBI
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
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 KeyInChIKey=UIUJIQZEACWQSV-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound 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
  • Hydrocarbon derivative
  • Organooxygen compound
  • Carbonyl group
  • Aldehyde
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Component of Butanoate metabolism
  • Component of Glutamate metabolism
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility194.0 mg/mLALOGPS
logP-0.47ALOGPS
logP-0.56ChemAxon
logS0.28ALOGPS
pKa (Strongest Acidic)4.13ChemAxon
pKa (Strongest Basic)-7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity22.61 m3·mol-1ChemAxon
Polarizability9.26 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-8e1cb554add6ed6c4e35View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9300000000-f5403e2e858fded273acView in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9000000000-bf9ebea7e4800559c111View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-000i-9200000000-e31b8066f970e1c178d1View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-052u-9000000000-76526f6c67d7a67c1d07View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0a4l-9000000000-70e85383e59b46bc429fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-0udi-5900000000-f1c2507387f92b6e1e3fView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-0zgi-9400000000-ba1b880a90d3ace8beeeView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-052f-9000000000-971836bb1672ad33106eView in MoNA
1D NMR1H NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location
  • Testes
Pathways
NameSMPDB LinkKEGG Link
2-Hydroxyglutric Aciduria (D And L Form)SMP00136Not Available
4-Hydroxybutyric Aciduria/Succinic Semialdehyde Dehydrogenase DeficiencySMP00243Not Available
Glutamate MetabolismSMP00072map00250
HomocarnosinosisSMP00385Not Available
Hyperinsulinism-Hyperammonemia SyndromeSMP00339Not Available
Succinic semialdehyde dehydrogenase deficiencySMP00567Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
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 ApplicableNot SpecifiedNot SpecifiedNormal details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022516
KNApSAcK IDNot Available
Chemspider ID1080
KEGG Compound IDC00232
BioCyc IDSUCC-S-ALD
BiGG ID34331
Wikipedia LinkSuccinic semialdehyde
NuGOwiki LinkHMDB01259
Metagene LinkHMDB01259
METLIN ID6114
PubChem Compound1112
PDB IDSSN
ChEBI ID16265
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. [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. [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. [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. [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. [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. [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. [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