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Showing metabocard for Succinic acid semialdehyde (HMDB0001259)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (4) Show 4 proteinsXML
enzymes (4) Show 4 proteins
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
MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0001259 (Succinic acid semialdehyde)


  Mrv1652309042000202D          

  7  6  0  0  0  0            999 V2000
 9999.6386 9999.3862    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.9237 9999.7980    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.2065 9999.3862    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.923710000.6237    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.3540 9999.7980    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.0676 9999.3862    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.7834 9999.7980    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  5  1  0  0  0  0
  2  3  1  0  0  0  0
  2  4  2  0  0  0  0
  5  6  1  0  0  0  0
  6  7  2  0  0  0  0
M  END
Download

3D MOL for HMDB0001259 (Succinic acid semialdehyde)

Download
3D SDF for HMDB0001259 (Succinic acid semialdehyde)

  Mrv1652309042000202D          

  7  6  0  0  0  0            999 V2000
 9999.6386 9999.3862    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.9237 9999.7980    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.2065 9999.3862    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.923710000.6237    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10000.3540 9999.7980    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.0676 9999.3862    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10001.7834 9999.7980    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  5  1  0  0  0  0
  2  3  1  0  0  0  0
  2  4  2  0  0  0  0
  5  6  1  0  0  0  0
  6  7  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0001259

> <DATABASE_NAME>
hmdb

> <SMILES>
OC(=O)CCC=O

> <INCHI_IDENTIFIER>
InChI=1S/C4H6O3/c5-3-1-2-4(6)7/h3H,1-2H2,(H,6,7)

> <INCHI_KEY>
UIUJIQZEACWQSV-UHFFFAOYSA-N

> <FORMULA>
C4H6O3

> <MOLECULAR_WEIGHT>
102.0886

> <EXACT_MASS>
102.031694058

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
13

> <JCHEM_AVERAGE_POLARIZABILITY>
9.263882313671203

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
4-oxobutanoic acid

> <ALOGPS_LOGP>
-0.47

> <JCHEM_LOGP>
-0.5570992196666666

> <ALOGPS_LOGS>
0.28

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
17.53992018087323

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.12725825924451

> <JCHEM_PKA_STRONGEST_BASIC>
-6.956114389966538

> <JCHEM_POLAR_SURFACE_AREA>
54.37

> <JCHEM_REFRACTIVITY>
22.611600000000003

> <JCHEM_ROTATABLE_BOND_COUNT>
3

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.94e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
succinic semialdehyde

> <JCHEM_VEBER_RULE>
0

$$$$

Download
3D-SDF for HMDB0001259 (Succinic acid semialdehyde)
https://cactus.nci.nih.gov/chemical/structure/OC(=O)CCC=O/file?format=sdf&get...
 OpenBabel09142113213D
Jmol version 14.31.45  2021-07-21 05:08 EXTRACT: ({0:12})
 13 12  0  0  0  0  0  0  0  0999 V2000
    2.6397   -0.7359    0.1478 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.5290    0.0071    0.0209 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6079    1.2126   -0.0025 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.1835   -0.6622   -0.0925 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9065    0.4032   -0.2257 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2519   -0.2662   -0.3391 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1377    0.0247    0.4285 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.4795   -0.2616    0.2171 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1712   -1.3066   -0.9716 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0007   -1.2610    0.7995 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8943    1.0475    0.6534 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7222    1.0019   -1.1177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4240   -1.0061   -1.1068 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  8  1  0  0  0  0
  2  3  2  0  0  0  0
  2  4  1  0  0  0  0
  4  5  1  0  0  0  0
  4  9  1  0  0  0  0
  4 10  1  0  0  0  0
  5  6  1  0  0  0  0
  5 11  1  0  0  0  0
  5 12  1  0  0  0  0
  6  7  2  0  0  0  0
  6 13  1  0  0  0  0
M  END
$$$$

Download
PDB for HMDB0001259 (Succinic acid semialdehyde)
HEADER    PROTEIN                                 04-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   04-SEP-20         0                                  
HETATM    1  C   UNK     0    8665.9928665.521   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8664.6588666.290   0.000  0.00  0.00           C+0
HETATM    3  O   UNK     0    8663.3198665.521   0.000  0.00  0.00           O+0
HETATM    4  O   UNK     0    8664.6588667.831   0.000  0.00  0.00           O+0
HETATM    5  C   UNK     0    8667.3278666.290   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0    8668.6608665.521   0.000  0.00  0.00           C+0
HETATM    7  O   UNK     0    8669.9968666.290   0.000  0.00  0.00           O+0
CONECT    1    2    5                                                       
CONECT    2    1    3    4                                                  
CONECT    3    2                                                            
CONECT    4    2                                                            
CONECT    5    1    6                                                       
CONECT    6    5    7                                                       
CONECT    7    6                                                            
MASTER        0    0    0    0    0    0    0    0    7    0   12    0
END

Download
3D PDB for HMDB0001259 (Succinic acid semialdehyde)
COMPND    https://cactus.nci.nih.gov/chemical/structure/OC(=O)CCC=O/file?format=sdf&get... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
HETATM    1  O   UNL     1       2.640  -0.736   0.148  1.00  0.00           O  
HETATM    2  C   UNL     1       1.529   0.007   0.021  1.00  0.00           C  
HETATM    3  O   UNL     1       1.608   1.213  -0.003  1.00  0.00           O  
HETATM    4  C   UNL     1       0.183  -0.662  -0.092  1.00  0.00           C  
HETATM    5  C   UNL     1      -0.906   0.403  -0.226  1.00  0.00           C  
HETATM    6  C   UNL     1      -2.252  -0.266  -0.339  1.00  0.00           C  
HETATM    7  O   UNL     1      -3.138   0.025   0.428  1.00  0.00           O  
HETATM    8  H   UNL     1       3.479  -0.262   0.217  1.00  0.00           H  
HETATM    9  H   UNL     1       0.171  -1.307  -0.972  1.00  0.00           H  
HETATM   10  H   UNL     1      -0.001  -1.261   0.799  1.00  0.00           H  
HETATM   11  H   UNL     1      -0.894   1.048   0.653  1.00  0.00           H  
HETATM   12  H   UNL     1      -0.722   1.002  -1.118  1.00  0.00           H  
HETATM   13  H   UNL     1      -2.424  -1.006  -1.107  1.00  0.00           H  
CONECT    1    2    8                                                 
CONECT    2    1    3    3    4                                       
CONECT    3    2    2                                                 
CONECT    4    2    5    9   10                                       
CONECT    5    4    6   11   12                                       
CONECT    6    5    7    7   13                                       
CONECT    7    6    6                                                 
CONECT    8    1                                                      
CONECT    9    4                                                      
CONECT   10    4                                                      
CONECT   11    5                                                      
CONECT   12    5                                                      
CONECT   13    6                                                      
MASTER        0    0    0    0    0    0    0    0   13    0   13    0
END

Download
SMILES for HMDB0001259 (Succinic acid semialdehyde)
OC(=O)CCC=O
Download
INCHI for HMDB0001259 (Succinic acid semialdehyde)
InChI=1S/C4H6O3/c5-3-1-2-4(6)7/h3H,1-2H2,(H,6,7)
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View in JSmolView Stereo Labels
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
Disposition
Route of exposure:
Source:
Biological location:
Process
Naturally occurring process:
Role
Indirect biological role:
Industrial application:
Biological role:
Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility194 g/LALOGPS
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 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 Spectral Properties
Collision Cross Sections
NameAdductTypeData SourceValueReference
DarkChem[M+H]+PredictedNot Available120.53531661259  
DarkChem[M-H]-PredictedNot Available114.69631661259  
AllCCS[M+H]+PredictedNot Available125.68532859911  
AllCCS[M-H]-PredictedNot Available124.04832859911  
Retention Indices
Underivatized
Not Available
Derivatized
DerivativeValueReference
Succinic acid semialdehyde,1TMS,#11080.5588https://arxiv.org/abs/1905.12712
Succinic acid semialdehyde,1TMS,#21203.3324https://arxiv.org/abs/1905.12712
Succinic acid semialdehyde,1TBDMS,#11315.5427https://arxiv.org/abs/1905.12712
Succinic acid semialdehyde,1TBDMS,#21441.5508https://arxiv.org/abs/1905.12712
Spectra
GC-MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-8e1cb554add6ed6c4e352014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9300000000-f5403e2e858fded273ac2014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-000i-9400000000-8e1cb554add6ed6c4e352017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-000i-9300000000-f5403e2e858fded273ac2017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-eb52f4d003b7d5b86b3d2017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-000i-9600000000-463f366ebd0be24a02832017-09-12View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-052f-9000000000-d9618eb947af434dcf2f2016-09-22View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-05fr-9800000000-40373963cdda851ebfea2017-10-06View Spectrum
MSMass Spectrum (Electron Ionization)splash10-052f-9100000000-b21c614ba2ea4ee4f4402021-09-05View Spectrum
MSMass Spectrum (Electron Ionization)splash10-0a4i-8900000000-5b42adf83e422cffefb62021-09-18View Spectrum
MSMass Spectrum (Electron Ionization)splash10-0a4i-9800000000-24fa3ff739ff064312ab2021-09-18View Spectrum
LC-MS/MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9000000000-bf9ebea7e4800559c1112012-07-24View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, negativesplash10-0udi-0900000000-64b3498f7cdd4597da6a2020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 1V, negativesplash10-0udi-1900000000-9d47aab2400f1da945bc2020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, negativesplash10-0udi-1900000000-94e1ac309574efbe39032020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 2V, negativesplash10-0udi-3900000000-7ec96b67f820176c39d32020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 3V, negativesplash10-0udi-5900000000-7708b3a1c05b69363aa92020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - Orbitrap 3V, negativesplash10-0zfr-8900000000-12726b727f621ac971b72020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum - n/a 7V, negativesplash10-0089-9000000000-9eb7dd16da8275702c752020-07-22View Spectrum
LC-MS/MSLC-MS/MS Spectrum -  35V, Negativesplash10-0udi-1900000000-567d38fac821ab993d192021-09-20View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-000i-9200000000-e31b8066f970e1c178d12015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Positivesplash10-052u-9000000000-76526f6c67d7a67c1d072015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Positivesplash10-0a4l-9000000000-70e85383e59b46bc429f2015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-0udi-5900000000-f1c2507387f92b6e1e3f2015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-0zgi-9400000000-ba1b880a90d3ace8beee2015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-052f-9000000000-971836bb1672ad33106e2015-05-27View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-052f-9000000000-06109e02fc0baa708cdc2021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Positivesplash10-052f-9000000000-ce51d68c1c700db8f9662021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Positivesplash10-0006-9000000000-d171f37acc222eeea0752021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-0a4i-9100000000-b23feac6075bc2594f5e2021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-0a4i-9000000000-aa34fb8bd9988d7944312021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-052f-9000000000-101491882eb1be877e892021-09-07View Spectrum
NMR
Spectrum TypeDescriptionDeposition DateView
1D NMR1H NMR Spectrum (1D, 600 MHz, H2O, experimental)2012-12-04View Spectrum
2D NMR[1H, 13C] NMR Spectrum (2D, 600 MHz, H2O, predicted)2012-12-05View 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
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
  Enzyme Details
1. 4-aminobutyrate aminotransferase, mitochondrial
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
  Enzyme Details
2. Succinate-semialdehyde dehydrogenase, mitochondrial
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
  Enzyme Details
3. Aflatoxin B1 aldehyde reductase member 2
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
  Enzyme Details
4. Hydroxyacid-oxoacid transhydrogenase, mitochondrial
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