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Showing metabocard for Pyruvic acid (HMDB0000243)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (53)transporters (6) Show 59 proteinsXML
enzymes (53)transporters (6) Show 59 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2021-09-07 16:24:13 UTC
HMDB IDHMDB0000243
Secondary Accession Numbers
  • HMDB00243
Metabolite Identification
Common NamePyruvic acid
DescriptionPyruvic acid or pyruvate is a simple alpha-keto acid. It is a three-carbon molecule containing a carboxylic acid group and a ketone functional group. Pyruvate is the simplest alpha-keto acid and according to official nomenclature by IUPAC, it is called alpha-keto propanoic acid. Like other keto acids, pyruvic acid can tautomerize from its ketone form to its enol form, containing a double bond and an alcohol. Pyruvate is found in all living organisms ranging from bacteria to plants to humans. It is intermediate compound in the metabolism of carbohydrates, proteins, and fats. Pyruvate is a key intermediate in several metabolic pathways throughout the cell. In particular, pyruvic acid can be made from glucose through glycolysis, converted back to carbohydrates (such as glucose) via gluconeogenesis, or to fatty acids through a reaction with acetyl-CoA. Pyruvic acid supplies energy to cells through the citric acid cycle (TCA or Krebs cycle) when oxygen is present (aerobic respiration), and alternatively ferments to produce lactate when oxygen is lacking (lactic acid). In glycolysis, phosphoenolpyruvate (PEP) is converted to pyruvate by pyruvate kinase. This reaction is strongly exergonic and irreversible. In gluconeogenesis, it takes two enzymes, pyruvate carboxylase and PEP carboxykinase, to catalyze the reverse transformation of pyruvate to PEP. Pyruvic acid is also a metabolite of Corynebacterium (PMID: 27872963 ).
Structure
Data?1582752118
MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000243 (Pyruvic acid)


  Mrv1652305271900282D          

  6  5  0  0  0  0            999 V2000
    2.0928    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2361   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  6  1  0  0  0  0
  2  4  2  0  0  0  0
  3  6  2  0  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
M  END
Download

3D MOL for HMDB0000243 (Pyruvic acid)

Download
3D SDF for HMDB0000243 (Pyruvic acid)

  Mrv1652305271900282D          

  6  5  0  0  0  0            999 V2000
    2.0928    0.2063    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -1.0312    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.5217    0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2361   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8072   -0.2063    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  6  1  0  0  0  0
  2  4  2  0  0  0  0
  3  6  2  0  0  0  0
  4  5  1  0  0  0  0
  4  6  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000243

> <DATABASE_NAME>
hmdb

> <SMILES>
CC(=O)C(O)=O

> <INCHI_IDENTIFIER>
InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)

> <INCHI_KEY>
LCTONWCANYUPML-UHFFFAOYSA-N

> <FORMULA>
C3H4O3

> <MOLECULAR_WEIGHT>
88.0621

> <EXACT_MASS>
88.016043994

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
10

> <JCHEM_AVERAGE_POLARIZABILITY>
7.310505872988212

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-oxopropanoic acid

> <ALOGPS_LOGP>
-0.38

> <JCHEM_LOGP>
0.06587395333333337

> <ALOGPS_LOGS>
0.18

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA_STRONGEST_ACIDIC>
2.93012330309767

> <JCHEM_PKA_STRONGEST_BASIC>
-9.583170381128017

> <JCHEM_POLAR_SURFACE_AREA>
54.37

> <JCHEM_REFRACTIVITY>
17.9897

> <JCHEM_ROTATABLE_BOND_COUNT>
1

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.34e+02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
pyruvic acid

> <JCHEM_VEBER_RULE>
0

$$$$

Download
3D-SDF for HMDB0000243 (Pyruvic acid)
https://cactus.nci.nih.gov/chemical/structure/CC(=O)C(O)=O/file?format=sdf&ge...
 OpenBabel09142113213D
Jmol version 14.31.45  2021-07-21 05:08 EXTRACT: ({0:9})
 10  9  0  0  0  0  0  0  0  0999 V2000
    1.7796    0.9097    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7457   -0.1867   -0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0923   -1.3437   -0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7050    0.1568   -0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6283   -0.8224    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0529    1.3185   -0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.0289    1.1740   -1.0277 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3814    1.7846    0.5139 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6764    0.5634    0.5139 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5563   -0.5507   -0.0000 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  0  0  0  0
  1  8  1  0  0  0  0
  1  9  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  6  2  0  0  0  0
  5 10  1  0  0  0  0
M  END
$$$$

Download
PDB for HMDB0000243 (Pyruvic acid)
HEADER    PROTEIN                                 27-MAY-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   27-MAY-19         0                                  
HETATM    1  O   UNK     0       3.907   0.385   0.000  0.00  0.00           O+0
HETATM    2  O   UNK     0       6.574   1.925   0.000  0.00  0.00           O+0
HETATM    3  O   UNK     0       5.240  -1.925   0.000  0.00  0.00           O+0
HETATM    4  C   UNK     0       6.574   0.385   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       7.907  -0.385   0.000  0.00  0.00           C+0
HETATM    6  C   UNK     0       5.240  -0.385   0.000  0.00  0.00           C+0
CONECT    1    6                                                            
CONECT    2    4                                                            
CONECT    3    6                                                            
CONECT    4    2    5    6                                                  
CONECT    5    4                                                            
CONECT    6    1    3    4                                                  
MASTER        0    0    0    0    0    0    0    0    6    0   10    0
END

Download
3D PDB for HMDB0000243 (Pyruvic acid)
COMPND    https://cactus.nci.nih.gov/chemical/structure/CC(=O)C(O)=O/file?format=sdf&ge... 
AUTHOR    GENERATED BY OPEN BABEL 3.1.0
HETATM    1  C   UNL     1       1.780   0.910   0.000  1.00  0.00           C  
HETATM    2  C   UNL     1       0.746  -0.187   0.000  1.00  0.00           C  
HETATM    3  O   UNL     1       1.092  -1.344   0.000  1.00  0.00           O  
HETATM    4  C   UNL     1      -0.705   0.157   0.000  1.00  0.00           C  
HETATM    5  O   UNL     1      -1.628  -0.822   0.000  1.00  0.00           O  
HETATM    6  O   UNL     1      -1.053   1.319   0.000  1.00  0.00           O  
HETATM    7  H   UNL     1       2.029   1.174  -1.028  1.00  0.00           H  
HETATM    8  H   UNL     1       1.381   1.785   0.514  1.00  0.00           H  
HETATM    9  H   UNL     1       2.676   0.563   0.514  1.00  0.00           H  
HETATM   10  H   UNL     1      -2.556  -0.551   0.000  1.00  0.00           H  
CONECT    1    2    7    8    9                                       
CONECT    2    1    3    3    4                                       
CONECT    3    2    2                                                 
CONECT    4    2    5    6    6                                       
CONECT    5    4   10                                                 
CONECT    6    4    4                                                 
CONECT    7    1                                                      
CONECT    8    1                                                      
CONECT    9    1                                                      
CONECT   10    5                                                      
MASTER        0    0    0    0    0    0    0    0   10    0   10    0
END

Download
SMILES for HMDB0000243 (Pyruvic acid)
CC(=O)C(O)=O
Download
INCHI for HMDB0000243 (Pyruvic acid)
InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)
Download
View in JSmolView Stereo Labels
Synonyms
ValueSource
2-Ketopropionic acidChEBI
2-Oxopropanoic acidChEBI
2-OxopropansaeureChEBI
2-OxopropionsaeureChEBI
Acetylformic acidChEBI
Acide pyruviqueChEBI
alpha-Ketopropionic acidChEBI
alpha-OxopropionsaeureChEBI
BrenztraubensaeureChEBI
BTSChEBI
CH3COCOOHChEBI
Pyroracemic acidChEBI
2-OxopropanoateKegg
2-KetopropionateGenerator
AcetylformateGenerator
a-KetopropionateGenerator
a-Ketopropionic acidGenerator
alpha-KetopropionateGenerator
Α-ketopropionateGenerator
Α-ketopropionic acidGenerator
a-OxopropionsaeureGenerator
Α-oxopropionsaeureGenerator
PyroracemateGenerator
PyruvateGenerator
2-OxopropionateHMDB
2-Oxopropionic acidHMDB
Acid, pyruvicHMDB
alpha-Ketopropanoic acidHMDB
Α-ketopropanoic acidHMDB
Pyruvic acidHMDB
Chemical FormulaC3H4O3
Average Molecular Weight88.0621
Monoisotopic Molecular Weight88.016043994
IUPAC Name2-oxopropanoic acid
Traditional Namepyruvic acid
CAS Registry Number127-17-3
SMILES
CC(=O)C(O)=O
InChI Identifier
InChI=1S/C3H4O3/c1-2(4)3(5)6/h1H3,(H,5,6)
InChI KeyLCTONWCANYUPML-UHFFFAOYSA-N
Chemical Taxonomy
Description Belongs to the class of organic compounds known as alpha-keto acids and derivatives. These are organic compounds containing an aldehyde substituted with a keto group on the adjacent carbon.
KingdomOrganic compounds  
Super ClassOrganic acids and derivatives  
ClassKeto acids and derivatives  
Sub ClassAlpha-keto acids and derivatives  
Direct ParentAlpha-keto acids and derivatives  
Alternative Parents
Substituents
  • Alpha-keto acid
    • 

  • Alpha-hydroxy ketone
    • 

  • Ketone
    • 

  • Monocarboxylic acid or derivatives
    • 

  • Carboxylic acid
    • 

  • Carboxylic acid derivative
    • 

  • Organic oxygen compound
    • 

  • Organic oxide
    • 

  • Hydrocarbon derivative
    • 

  • Organooxygen compound
    • 

  • Carbonyl group
    • 

  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
Physiological effect
Health effect:
Disposition
Route of exposure:
Source:
Biological location:
Process
Naturally occurring process:
Role
Industrial application:
Biological role:
Physical Properties
StateLiquid
Experimental Properties
PropertyValueReference
Melting Point13.8 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility1000 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility134 g/LALOGPS
logP-0.38ALOGPS
logP0.066ChemAxon
logS0.18ALOGPS
pKa (Strongest Acidic)2.93ChemAxon
pKa (Strongest Basic)-9.6ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count1ChemAxon
Refractivity17.99 m³·mol⁻¹ChemAxon
Polarizability7.31 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Spectra
GC-MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-00dr-4900000000-f26ef76666e40ab9fe612014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-00di-5900000000-b8e81f82572d4796e9442014-06-16View Spectrum
GC-MSGC-MS Spectrum - GC-MS (2 TMS)splash10-014i-5970000000-154bf9ad168a12593fcc2014-06-16View Spectrum
GC-MSGC-MS Spectrum - EI-B (Non-derivatized)splash10-0006-9000000000-a2cf85a5e1d2379d26df2017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dr-4900000000-f26ef76666e40ab9fe612017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-00di-5900000000-b8e81f82572d4796e9442017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-MS (Non-derivatized)splash10-014i-5970000000-154bf9ad168a12593fcc2017-09-12View Spectrum
GC-MSGC-MS Spectrum - GC-EI-TOF (Non-derivatized)splash10-00dr-5900000000-5b1f470d4ff91420618c2017-09-12View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positivesplash10-0006-9000000000-5417b44aa241a7ba27e82016-09-22View Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positivesplash10-00dm-9400000000-6db65a709bdc47e3adf72017-10-06View Spectrum
MSMass Spectrum (Electron Ionization)splash10-0006-9000000000-f315d0752893e7d0c6572014-09-20View Spectrum
LC-MS/MS
Spectrum TypeDescriptionSplash KeyDeposition DateView
LC-MS/MSLC-MS/MS Spectrum - EI-B (HITACHI M-80B) , Positivesplash10-0006-9000000000-a2cf85a5e1d2379d26df2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-000i-9000000000-dd49835da8355fb6e6252012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-000i-9000000000-f09d8e3d7a774b255d892012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-0006-9000000000-7d91f6f626cab1a366fd2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0006-9000000000-8ae98cdb3e142034e52a2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 50V, Negativesplash10-0006-9000000000-e04e6c68013983e1b6dc2012-08-31View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-9000000000-dd49835da8355fb6e6252017-09-14View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-000i-9000000000-f09d8e3d7a774b255d892017-09-14View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-7d91f6f626cab1a366fd2017-09-14View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-8ae98cdb3e142034e52a2017-09-14View Spectrum
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-e04e6c68013983e1b6dc2017-09-14View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-000i-9000000000-d0defa72b09503c6d6d12016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Positivesplash10-000f-9000000000-c25fa150e9c490319a2a2016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Positivesplash10-0006-9000000000-ccb42b4c05ddd001990f2016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-000i-9000000000-faf36ff70d62053702702016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-000i-9000000000-60c1a02aabf80f51050f2016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-000f-9000000000-ca5f4a2f06787d8b62a02016-09-12View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Positivesplash10-0006-9000000000-0eb1fb2cdd24bdc786012021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Positivesplash10-0006-9000000000-87bbaed151efac0845912021-09-06View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  10V, Negativesplash10-000i-9000000000-0ff17f97b83100ef5b2c2021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  20V, Negativesplash10-000f-9000000000-3732177135731d084b0e2021-09-07View Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum -  40V, Negativesplash10-0006-9000000000-d154cbf253a17855bcd72021-09-07View Spectrum
NMR
Spectrum TypeDescriptionDeposition DateView
1D NMR13C NMR Spectrum (1D, 400 MHz, H2O, experimental)2012-12-04View Spectrum
1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04View Spectrum
1D NMR1H NMR Spectrum (1D, 300 MHz, CDCl3, experimental)2014-09-20View Spectrum
1D NMR13C NMR Spectrum (1D, 25.16 MHz, CDCl3, experimental)2014-09-23View Spectrum
1D NMR1H NMR Spectrum (1D, D2O, experimental)2016-10-22View Spectrum
1D NMR1H NMR Spectrum (1D, D2O, experimental)2016-10-22View Spectrum
1D NMR1H NMR Spectrum (1D, D2O, experimental)2016-10-22View Spectrum
1D NMR13C NMR Spectrum (1D, D2O, experimental)2016-10-22View Spectrum
2D NMR[1H, 13C] NMR Spectrum (2D, 600 MHz, H2O, predicted)2012-12-05View Spectrum
Biological Properties
Cellular Locations
  • Extracellular
    • 

  • Mitochondria
    • 

  • Peroxisome
Biospecimen Locations
  • Blood
    • 

  • Breast Milk
    • 

  • Cellular Cytoplasm
    • 

  • Cerebrospinal Fluid (CSF)
    • 

  • Feces
    • 

  • Saliva
    • 

  • Sweat
    • 

  • Urine
Tissue Locations
  • Adipose Tissue
    • 

  • Brain
    • 

  • Fibroblasts
    • 

  • Heart
    • 

  • Kidney
    • 

  • Liver
    • 

  • Neuron
    • 

  • Pancreas
    • 

  • Placenta
    • 

  • Skeletal Muscle
    • 

  • Spleen
    • 

  • Testis
    • 

  • Thyroid Gland
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified34.5 +/- 25.2 uMAdult (>18 years old)BothNormal  details
BloodDetected and Quantified<150 uMNewborn (0-30 days old)Not SpecifiedNormal  details
BloodDetected and Quantified<200 uMInfant (0-1 year old)Not SpecifiedNormal  details
BloodDetected and Quantified64 (22-258) uMAdult (>18 years old)BothNormal  details
BloodDetected and Quantified100 (50-190) uMNot SpecifiedNot Specified
 
Normal		  details
BloodDetected and Quantified56.778 uMInfant (0-1 year old)Not SpecifiedNormal  details
BloodDetected and Quantified81.0 +/- 34.0 uMNewborn (0-30 days old)BothNormal
    • Geigy Scientific ...
  details
BloodDetected and Quantified76.0 +/- 10.0 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
  details
BloodDetected and Quantified63.0 +/- 25.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
  details
BloodDetected and Quantified40-100 uMNot SpecifiedNot SpecifiedNormal  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal  details
BloodDetected and Quantified75.5 (10.0-141.0) uMNewborn (0-30 days old)Not SpecifiedNormal  details
BloodDetected and Quantified50-140 uMNot SpecifiedNot SpecifiedNormal  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
BloodDetected and Quantified40-70 uMInfant (0-1 year old)Both
 
Normal		  details
BloodDetected and Quantified<200 uMChildren (1-13 years old)Not SpecifiedNormal  details
BloodDetected and Quantified22.00-258.0 uMAdult (>18 years old)BothNormal  details
BloodDetected and Quantified64 (28-145) uMAdult (>18 years old)MaleNormal  details
BloodDetected and Quantified64 (29-144) uMAdult (>18 years old)FemaleNormal  details
BloodDetected and Quantified67 (27-164) uMAdult (>18 years old)BothNormal  details
BloodDetected and Quantified64 (29-144) uMAdult (>18 years old)BothNormal  details
BloodDetected and Quantified66 (34-117) uMElderly (>65 years old)BothNormal  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
Breast MilkDetected and Quantified5.7 +/- 3.3 uMAdult (>18 years old)Female
 
Normal		  details
Cellular CytoplasmDetected and Quantified77.0 (27.0-127) uMAdult (>18 years old)BothNormal  details
Cellular CytoplasmDetected and Quantified360 (350-370) uMAdult (>18 years old)BothNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified153.0 (121.0-185.0) uMAdult (>18 years old)BothNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified27 +/- 11 uMChildren (1 - 13 years old)Not SpecifiedNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified30 -130 uMInfant (0-1 year old)MaleNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified53 +/- 42 uMAdult (>18 years old)BothNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified71.0 +/- 31.0 uMAdult (>18 years old)Not SpecifiedNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified50-140 uMNot SpecifiedNot SpecifiedNormal  details
Cerebrospinal Fluid (CSF)Detected and Quantified115.0 +/- 17.0 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
  details
Cerebrospinal Fluid (CSF)Detected and Quantified136.0 +/- 29.0 uMChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
FecesDetected and Quantified13361.783 (3633.799-23089.767) nmol/g wet fecesInfant (0-1 year old)Not Specified
 
Normal		  details
FecesDetected and Quantified27783.424 (17336.251-38230.597) nmol/g wet fecesInfant (0-1 year old)Not Specified
 
Normal		  details
FecesDetected and Quantified62607.334 (33271.975-91942.693) nmol/g wet fecesInfant (0-1 year old)Not Specified
 
Normal		  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified43.3 +/- 65.1 uMAdult (>18 years old)Female
 
Normal
    • Sugimoto et al. (...
  details
SalivaDetected and Quantified62.5 +/- 47.1 uMAdult (>18 years old)Female
 
Normal
    • Sugimoto et al. (...
  details
SalivaDetected and Quantified9 (<1-78) uMAdult (>18 years old)Male
 
Normal		  details
SalivaDetected and Quantified32 (<1-849) uMAdult (>18 years old)Male
 
Normal		  details
SalivaDetected and Quantified68.6 +/- 69.1 uMAdult (>18 years old)Female
 
Normal
    • Sugimoto et al. (...
  details
SalivaDetected and Quantified36 (9-395) uMAdult (>18 years old)Male
 
Normal		  details
SalivaDetected and Quantified15 (7-91) uMAdult (>18 years old)Female
 
Normal		  details
SalivaDetected and Quantified350 +/- 740 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified2310 +/- 3470 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected and Quantified1830 +/- 2960 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Male
 
Normal		  details
SalivaDetected and Quantified470 +/- 610 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified410 +/- 1000 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified16.0 +/- 18.3 uMAdult (>18 years old)Male
 
Normal
    • Sugimoto et al. (...
  details
SalivaDetected and Quantified290 +/- 620 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified200 +/- 330 uMAdult (>18 years old)Both
 
Normal		  details
SalivaDetected and Quantified5350 (100-10600) uMAdult (>18 years old)BothNormal  details
SalivaDetected and Quantified107.45 +/- 64.52 uMAdult (>18 years old)FemaleNormal  details
SalivaDetected and Quantified1-128 uMAdult (>18 years old)Male
 
normal		  details
SalivaDetected and Quantified1-76 uMAdult (>18 years old)Male
 
normal		  details
SalivaDetected and Quantified85.38 +/- 94.78 uMAdult (>18 years old)BothNormal
    • Zerihun T. Dame, ...
  details
SweatDetected but not QuantifiedNot QuantifiedAdult BothNormal  details
UrineDetected and Quantified0-30 umol/mmol creatinineChildren (1-13 years old)Not SpecifiedNormal  details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal  details
UrineDetected and Quantified19.55-89.06 umol/mmol creatinineNewborn (0-30 days old)BothNormal
    • López Hernández Y...
  details
UrineDetected and Quantified33.86(23.57-47.66) umol/mmol creatinineNewborn (0-30 days old)FemaleNormal
    • López Hernández Y...
  details
UrineDetected and Quantified34.18(19.29-92.98) umol/mmol creatinineNewborn (0-30 days old)MaleNormal
    • López Hernández Y...
  details
UrineDetected and Quantified9.79 +/- 9.34 umol/mmol creatinineInfant (0-1 year old)Not SpecifiedNormal  details
UrineDetected and Quantified2.1 (1.0-3.7) umol/mmol creatinineAdult (>18 years old)Both
 
Normal		  details
UrineDetected and Quantified14-28.0 umol/mmol creatinineInfant (0-1 year old)Male
 
Normal		  details
UrineDetected and Quantified14.0-23.6 umol/mmol creatinineInfant (0-1 year old)Female
 
Normal		  details
UrineDetected and Quantified16.9-30.6 umol/mmol creatinineInfant (0-1 year old)Male
 
Normal		  details
UrineDetected and Quantified2.27-79.53 umol/mmol creatinineAdult (>18 years old)BothNormal
    • David F. Putnam C...
  details
UrineDetected and Quantified17.3-35.1 umol/mmol creatinineInfant (0-1 year old)Female
 
Normal		  details
UrineDetected and Quantified4.21 +/- 1.37 umol/mmol creatinineChildren (1-13 years old)BothNormal
    • Geigy Scientific ...
  details
UrineDetected and Quantified8.349 +/- 4.704 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
 
Normal
    • Mordechai, Hien, ...
  details
UrineDetected and Quantified1.7985 (0-3.597) umol/mmol creatinineAdult (>18 years old)BothNormal  details
UrineDetected and Quantified5.4 (2.6-7.9) umol/mmol creatinineAdult (>18 years old)BothNormal  details
UrineDetected and Quantified2.7 (0.8-6.4) umol/mmol creatinineAdult (>18 years old)Both
 
Normal		  details
UrineDetected and Quantified21.2 (8.8-59.3) umol/mmol creatinineNewborn (0-30 days old)BothNormal  details
UrineDetected and Quantified51.865 +/- 15.608 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
 
Normal
    • Mordechai, Hien, ...
  details
UrineDetected and Quantified28.6 (12.9-43.0) umol/mmol creatinineInfant (0-1 year old)BothNormal  details
UrineDetected and Quantified12.4 (5.3-22.1) umol/mmol creatinineChildren (1-13 years old)BothNormal  details
UrineDetected and Quantified10.2 (4.7-40.5) umol/mmol creatinineAdolescent (13-18 years old)BothNormal  details
UrineDetected and Quantified38.50 (18.32 – 69.64)  umol/mmol creatinineAdult (>18 years old)BothNormal
    • Lorena Ivona ŞTEF...
  details
UrineDetected and Quantified2.13 (0.54-8.67) umol/mmol creatinineAdult (>18 years old)MaleNormal  details
UrineDetected and Quantified1.68 (0.62-3.30) umol/mmol creatinineAdult (>18 years old)FemaleNormal  details
UrineDetected and Quantified7 umol/mmol creatinineAdult (>18 years old)BothNormal  details
UrineDetected and Quantified13-41 umol/mmol creatinineNewborn (0-30 days old)Both
 
Normal		  details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified77.9 (32.4)  uMAdult (>18 years old)FemaleDown syndrome pregnancy  details
BloodDetected and Quantified61.0 (26.4)  uMAdult (>18 years old)FemalePregnancy  details
BloodDetected and Quantified150 (120-170) uMAdult (>18 years old)Both
 
Myopathy with lactic acidosis, hereditary		  details
BloodDetected and Quantified500 uMInfant (0-1 year old)Female
 
Fructose-1,6-bisphosphatase deficiency		  details
BloodDetected and Quantified1090 (650-1520) uMAdult (>18 years old)Both
 
Myopathy with lactic acidosis, hereditary		  details
BloodDetected and Quantified710 uMAdult (>18 years old)Both
 
Myopathy with lactic acidosis, hereditary		  details
BloodDetected and Quantified800-900 uMNewborn (0-30 days old)FemaleMitochondrial pyruvate carrier deficiency  details
BloodDetected and Quantified670 +/- 990 uMChildren (1-13 years old)BothPyruvate dehydrogenase deficiency (E1)  details
BloodDetected and Quantified560 +/- 830 uMChildren (1-13 years old)BothPyruvate dehydrogenase deficiency E1-alpha  details
BloodDetected and Quantified400 uMInfant (0-1 year old)MalePyruvate dehydrogenase phosphatase deficiency  details
BloodDetected and Quantified50.2 +/- 40 uMAdult (>18 years old)BothHeart Transplant  details
BloodDetected and Quantified60-240 uMInfant (0-1 year old)Male
 
2-Ketoglutarate dehydrogenase complex deficiency		  details
BloodDetected and Quantified499.647 uMInfant (0-1 year old)MaleLeigh Syndrome  details
BloodDetected and Quantified74.66 +/- 35.11  uMAdult (>18 years old)Female
 
Pregnancy with fetuses with trisomy 18		  details
BloodDetected and Quantified59.70 +/- 23.3  uMAdult (>18 years old)FemalePregnancy  details
BloodDetected and Quantified500 uMInfant (0-1 year old)Male
 
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency		  details
BloodDetected and Quantified89.3 (27.0)  uMAdult (>18 years old)FemaleEarly preeclampsia  details
BloodDetected and Quantified400-40200 uMNewborn (0-30 days old)Both
 
3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency		  details
BloodDetected and Quantified71.3 (26.7) uMAdult (>18 years old)FemalePregnancy  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer  details
BloodDetected and Quantified102-230 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
gastric cancer		  details
BloodDetected and Quantified270 uMNewborn (0-30 days old)Male2-Ketoglutarate dehydrogenase complex deficiency  details
BloodDetected and Quantified5-440 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Colorectal cancer		  details
BloodDetected and Quantified63.92 (19.24) uMAdult (>18 years old)FemalePregnancy with fetus having congenital heart defect  details
BloodDetected and Quantified54.52 (25.5)  uMAdult (>18 years old)FemalePregnancy  details
BloodDetected and Quantified83.1 (45.8)  uMAdult (>18 years old)FemaleLate-onset preeclampsia  details
BloodDetected and Quantified62.1 (24.1)  uMAdult (>18 years old)FemalePregnancy  details
BloodDetected and Quantified120(80-160) uMInfant (0-1 year old)Both
 
GRACILE syndrome		  details
BloodDetected and Quantified160-320 uMInfant (0-1 year old)MaleDihydrolipoamide Dehydrogenase Deficiency  details
BloodDetected and Quantified440 uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency  details
BloodDetected and Quantified93.5 (27.0-160.0) uMAdult (>18 years old)Both3-methylglutaconic aciduria type II, X-linked
    • MetaGene: Metabol...
  details
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Schizophrenia		  details
BloodDetected and Quantified313 +/- 31 uMInfant (0-1 year old)Male
 
Glucagon deficiency		  details
BloodDetected and Quantified60 uMChildren (1-13 years old)Male
 
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency		  details
Cellular CytoplasmDetected and Quantified140 (130-150) uMAdult (>18 years old)BothAnoxia  details
Cerebrospinal Fluid (CSF)Detected and Quantified195.0 (175.0-215.0) uMAdult (>18 years old)Both
 
Meningitis		  details
Cerebrospinal Fluid (CSF)Detected and Quantified49 uMChildren (1 - 13 years old)Not SpecifiedIsolated 3-Methylcrotonyl-CoA Carboxylase Deficiency  details
Cerebrospinal Fluid (CSF)Detected and Quantified253 - 345 uMInfant (0-1 year old)MaleFumarase deficiency  details
Cerebrospinal Fluid (CSF)Detected and Quantified570 +/- 400 uMChildren (1-13 years old)BothPyruvate dehydrogenase deficiency (E1)  details
Cerebrospinal Fluid (CSF)Detected and Quantified480 +/- 270 uMChildren (1-13 years old)BothPyruvate dehydrogenase deficiency E1-alpha  details
Cerebrospinal Fluid (CSF)Detected and Quantified176.900-191.400 uMChildren (1-13 years old)Both2-Ketoglutarate dehydrogenase complex deficiency  details
Cerebrospinal Fluid (CSF)Detected and Quantified360 uMChildren (1-13 years old)MaleLipoyltransferase 1 Deficiency  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Colorectal cancer		  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer  details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)Both
 
Irritable Bowel Syndrome		  details
SalivaDetected and Quantified149.14 +/- 172.29 uMAdult (>18 years old)MaleAlzheimer's disease  details
SalivaDetected and Quantified72.05 +/- 29.52 uMAdult (>18 years old)MaleFrontotemporal lobe dementia  details
SalivaDetected and Quantified138.32 +/- 112.14 uMAdult (>18 years old)BothLewy body disease  details
UrineDetected and Quantified0.2 (0.10-0.30) umol/mmol creatinineChildren (1-13 years old)Both3-Methylglutaconic Aciduria (TYPE II), X-LINKED
    • MetaGene: Metabol...
  details
UrineDetected and Quantified400-1255 umol/mmol creatinineChildren (1-13 years old)BothPearson Syndrome  details
UrineDetected and Quantified9.988 +/- 10.859 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
 
Eosinophilic esophagitis
    • Mordechai, Hien, ...
  details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal cancer  details
UrineDetected and Quantified44.226 +/- 13.63 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
 
Eosinophilic esophagitis
    • Mordechai, Hien, ...
  details
UrineDetected and Quantified54.585 +/- 36.553 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
 
Gastroesophageal reflux disease
    • Mordechai, Hien, ...
  details
UrineDetected and Quantified45.58 (18.06 – 104.62) umol/mmol creatinineAdult (>18 years old)BothType 1 diabetes Mellitus
    • Lorena Ivona ŞTEF...
  details
Associated Disorders and Diseases
Disease References
3-methylglutaconic aciduria type II, X-linked
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium. 
Colorectal cancer
  1. Qiu Y, Cai G, Su M, Chen T, Zheng X, Xu Y, Ni Y, Zhao A, Xu LX, Cai S, Jia W: Serum metabolite profiling of human colorectal cancer using GC-TOFMS and UPLC-QTOFMS. J Proteome Res. 2009 Oct;8(10):4844-50. doi: 10.1021/pr9004162. [PubMed:19678709  ] 
  2. Ni Y, Xie G, Jia W: Metabonomics of human colorectal cancer: new approaches for early diagnosis and biomarker discovery. J Proteome Res. 2014 Sep 5;13(9):3857-70. doi: 10.1021/pr500443c. Epub 2014 Aug 14. [PubMed:25105552  ] 
  3. Sinha R, Ahn J, Sampson JN, Shi J, Yu G, Xiong X, Hayes RB, Goedert JJ: Fecal Microbiota, Fecal Metabolome, and Colorectal Cancer Interrelations. PLoS One. 2016 Mar 25;11(3):e0152126. doi: 10.1371/journal.pone.0152126. eCollection 2016. [PubMed:27015276  ] 
  4. Goedert JJ, Sampson JN, Moore SC, Xiao Q, Xiong X, Hayes RB, Ahn J, Shi J, Sinha R: Fecal metabolomics: assay performance and association with colorectal cancer. Carcinogenesis. 2014 Sep;35(9):2089-96. doi: 10.1093/carcin/bgu131. Epub 2014 Jul 18. [PubMed:25037050  ] 
Stomach cancer
  1. Ikeda A, Nishiumi S, Shinohara M, Yoshie T, Hatano N, Okuno T, Bamba T, Fukusaki E, Takenawa T, Azuma T, Yoshida M: Serum metabolomics as a novel diagnostic approach for gastrointestinal cancer. Biomed Chromatogr. 2012 May;26(5):548-58. doi: 10.1002/bmc.1671. Epub 2011 Jul 20. [PubMed:21773981  ] 
Schizophrenia
  1. Yang J, Chen T, Sun L, Zhao Z, Qi X, Zhou K, Cao Y, Wang X, Qiu Y, Su M, Zhao A, Wang P, Yang P, Wu J, Feng G, He L, Jia W, Wan C: Potential metabolite markers of schizophrenia. Mol Psychiatry. 2013 Jan;18(1):67-78. doi: 10.1038/mp.2011.131. Epub 2011 Oct 25. [PubMed:22024767  ] 
Early preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326  ] 
Pregnancy
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomics and first-trimester prediction of early-onset preeclampsia. J Matern Fetal Neonatal Med. 2012 Oct;25(10):1840-7. doi: 10.3109/14767058.2012.680254. Epub 2012 Apr 28. [PubMed:22494326  ] 
  2. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745  ] 
  3. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester Down syndrome prediction. Am J Obstet Gynecol. 2013 May;208(5):371.e1-8. doi: 10.1016/j.ajog.2012.12.035. Epub 2013 Jan 8. [PubMed:23313728  ] 
  4. Bahado-Singh RO, Akolekar R, Chelliah A, Mandal R, Dong E, Kruger M, Wishart DS, Nicolaides K: Metabolomic analysis for first-trimester trisomy 18 detection. Am J Obstet Gynecol. 2013 Jul;209(1):65.e1-9. doi: 10.1016/j.ajog.2013.03.028. Epub 2013 Mar 25. [PubMed:23535240  ] 
  5. Bahado-Singh RO, Ertl R, Mandal R, Bjorndahl TC, Syngelaki A, Han B, Dong E, Liu PB, Alpay-Savasan Z, Wishart DS, Nicolaides KH: Metabolomic prediction of fetal congenital heart defect in the first trimester. Am J Obstet Gynecol. 2014 Sep;211(3):240.e1-240.e14. doi: 10.1016/j.ajog.2014.03.056. Epub 2014 Apr 1. [PubMed:24704061  ] 
Late-onset preeclampsia
  1. Bahado-Singh RO, Akolekar R, Mandal R, Dong E, Xia J, Kruger M, Wishart DS, Nicolaides K: First-trimester metabolomic detection of late-onset preeclampsia. Am J Obstet Gynecol. 2013 Jan;208(1):58.e1-7. doi: 10.1016/j.ajog.2012.11.003. Epub 2012 Nov 13. [PubMed:23159745  ] 
Glucagon deficiency
  1. Vidnes J, Oyasaeter S: Glucagon deficiency causing severe neonatal hypoglycemia in a patient with normal insulin secretion. Pediatr Res. 1977 Sep;11(9 Pt 1):943-9. [PubMed:904979  ] 
2-Ketoglutarate dehydrogenase complex deficiency
  1. Bonnefont JP, Chretien D, Rustin P, Robinson B, Vassault A, Aupetit J, Charpentier C, Rabier D, Saudubray JM, Munnich A: Alpha-ketoglutarate dehydrogenase deficiency presenting as congenital lactic acidosis. J Pediatr. 1992 Aug;121(2):255-8. [PubMed:1640293  ] 
  2. Kohlschutter A, Behbehani A, Langenbeck U, Albani M, Heidemann P, Hoffmann G, Kleineke J, Lehnert W, Wendel U: A familial progressive neurodegenerative disease with 2-oxoglutaric aciduria. Eur J Pediatr. 1982 Feb;138(1):32-7. [PubMed:7075624  ] 
  3. Guffon N, Lopez-Mediavilla C, Dumoulin R, Mousson B, Godinot C, Carrier H, Collombet JM, Divry P, Mathieu M, Guibaud P: 2-Ketoglutarate dehydrogenase deficiency, a rare cause of primary hyperlactataemia: report of a new case. J Inherit Metab Dis. 1993;16(5):821-30. [PubMed:8295396  ] 
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
  1. Ozand PT, al Aqeel A, Gascon G, Brismar J, Thomas E, Gleispach H: 3-Hydroxy-3-methylglutaryl-coenzyme A (HMG-CoA) lyase deficiency in Saudi Arabia. J Inherit Metab Dis. 1991;14(2):174-88. [PubMed:1886403  ] 
3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency
  1. Morris AA, Lascelles CV, Olpin SE, Lake BD, Leonard JV, Quant PA: Hepatic mitochondrial 3-hydroxy-3-methylglutaryl-coenzyme a synthase deficiency. Pediatr Res. 1998 Sep;44(3):392-6. doi: 10.1203/00006450-199809000-00021. [PubMed:9727719  ] 
Leigh's syndrome, subacute necrotizing encephalopathy, SNE
  1. Hommes FA, Polman HA, Reerink JD: Leigh's encephalomyelopathy: an inborn error of gluconeogenesis. Arch Dis Child. 1968 Aug;43(230):423-6. [PubMed:4873809  ] 
Myopathy with lactic acidosis, hereditary
  1. LARSSON LE, LINDERHOLM H, MUELLER R, RINGQVIST T, SOERNAES R: HEREDITARY METABOLIC MYOPATHY WITH PAROXYSMAL MYOGLOBINURIA DUE TO ABNORMAL GLYCOLYSIS. J Neurol Neurosurg Psychiatry. 1964 Oct;27:361-80. [PubMed:14213465  ] 
Fructose-1,6-diphosphatase deficiency
  1. Pagliara AS, Karl IE, Keating JP, Brown BI, Kipnis DM: Hepatic fructose-1,6-diphosphatase deficiency. A cause of lactic acidosis and hypoglycemia in infancy. J Clin Invest. 1972 Aug;51(8):2115-23. doi: 10.1172/JCI107018. [PubMed:4341015  ] 
Mitochondrial pyruvate carrier deficiency
  1. Brivet M, Garcia-Cazorla A, Lyonnet S, Dumez Y, Nassogne MC, Slama A, Boutron A, Touati G, Legrand A, Saudubray JM: Impaired mitochondrial pyruvate importation in a patient and a fetus at risk. Mol Genet Metab. 2003 Mar;78(3):186-92. [PubMed:12649063  ] 
Pyruvate dehydrogenase deficiency (E1)
  1. Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW: The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients. Mol Genet Metab. 2012 Jan;105(1):34-43. doi: 10.1016/j.ymgme.2011.09.032. Epub 2011 Oct 7. [PubMed:22079328  ] 
Pyruvate dehydrogenase deficiency
  1. Patel KP, O'Brien TW, Subramony SH, Shuster J, Stacpoole PW: The spectrum of pyruvate dehydrogenase complex deficiency: clinical, biochemical and genetic features in 371 patients. Mol Genet Metab. 2012 Jan;105(1):34-43. doi: 10.1016/j.ymgme.2011.09.032. Epub 2011 Oct 7. [PubMed:22079328  ] 
Pyruvate dehydrogenase phosphatase deficiency
  1. Robinson BH, Sherwood WG: Pyruvate dehydrogenase phosphatase deficiency: a cause of congenital chronic lactic acidosis in infancy. Pediatr Res. 1975 Dec;9(12):935-9. doi: 10.1203/00006450-197512000-00015. [PubMed:172850  ] 
Dihydrolipoamide Dehydrogenase Deficiency
  1. Kuhara T, Shinka T, Inoue Y, Matsumoto M, Yoshino M, Sakaguchi Y, Matsumoto I: Studies of urinary organic acid profiles of a patient with dihydrolipoyl dehydrogenase deficiency. Clin Chim Acta. 1983 Sep 30;133(2):133-40. [PubMed:6688766  ] 
Lipoyltransferase 1 Deficiency
  1. Soreze Y, Boutron A, Habarou F, Barnerias C, Nonnenmacher L, Delpech H, Mamoune A, Chretien D, Hubert L, Bole-Feysot C, Nitschke P, Correia I, Sardet C, Boddaert N, Hamel Y, Delahodde A, Ottolenghi C, de Lonlay P: Mutations in human lipoyltransferase gene LIPT1 cause a Leigh disease with secondary deficiency for pyruvate and alpha-ketoglutarate dehydrogenase. Orphanet J Rare Dis. 2013 Dec 17;8:192. doi: 10.1186/1750-1172-8-192. [PubMed:24341803  ] 
GRACILE syndrome
  1. Visapaa I, Fellman V, Vesa J, Dasvarma A, Hutton JL, Kumar V, Payne GS, Makarow M, Van Coster R, Taylor RW, Turnbull DM, Suomalainen A, Peltonen L: GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1L. Am J Hum Genet. 2002 Oct;71(4):863-76. Epub 2002 Sep 5. [PubMed:12215968  ] 
Anoxia
  1. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834  ] 
Meningitis
  1. Subramanian A, Gupta A, Saxena S, Gupta A, Kumar R, Nigam A, Kumar R, Mandal SK, Roy R: Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children. NMR Biomed. 2005 Jun;18(4):213-25. [PubMed:15627241  ] 
3-Methyl-crotonyl-glycinuria
  1. de Kremer RD, Latini A, Suormala T, Baumgartner ER, Larovere L, Civallero G, Guelbert N, Paschini-Capra A, Depetris-Boldini C, Mayor CQ: Leukodystrophy and CSF purine abnormalities associated with isolated 3-methylcrotonyl-CoA carboxylase deficiency. Metab Brain Dis. 2002 Mar;17(1):13-8. [PubMed:11893004  ] 
Fumarase deficiency
  1. Tregoning S, Salter W, Thorburn DR, Durkie M, Panayi M, Wu JY, Easterbrook A, Coman DJ: Fumarase deficiency in dichorionic diamniotic twins. Twin Res Hum Genet. 2013 Dec;16(6):1117-20. doi: 10.1017/thg.2013.72. Epub 2013 Nov 4. [PubMed:24182348  ] 
Irritable bowel syndrome
  1. Hong YS, Hong KS, Park MH, Ahn YT, Lee JH, Huh CS, Lee J, Kim IK, Hwang GS, Kim JS: Metabonomic understanding of probiotic effects in humans with irritable bowel syndrome. J Clin Gastroenterol. 2011 May-Jun;45(5):415-25. doi: 10.1097/MCG.0b013e318207f76c. [PubMed:21494186  ] 
Alzheimer's disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013  Sep 6. [PubMed:23857558  ] 
Frontotemporal dementia
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013  Sep 6. [PubMed:23857558  ] 
Lewy body disease
  1. Tsuruoka M, Hara J, Hirayama A, Sugimoto M, Soga T, Shankle WR, Tomita M: Capillary electrophoresis-mass spectrometry-based metabolome analysis of serum and saliva from neurodegenerative dementia patients. Electrophoresis. 2013 Oct;34(19):2865-72. doi: 10.1002/elps.201300019. Epub 2013  Sep 6. [PubMed:23857558  ] 
Diabetes mellitus type 1
  1.  (). Lorena Ivona ŞTEFAN, Alina NICOLESCU, Simona POPA, Maria MOŢA, Eugenia KOVACS and Calin DELEANU. 1H-NMR URINE METABOLIC PROFILING IN TYPE 1 DIABETES MELLITUS. Rev. Roum. Chim., 2010, 55(11-12), 1033-1037 . . 
Eosinophilic esophagitis
  1.  (). Mordechai, Hien, and David S. Wishart. . 
Pearson Syndrome
  1. Crippa BL, Leon E, Calhoun A, Lowichik A, Pasquali M, Longo N: Biochemical abnormalities in Pearson syndrome. Am J Med Genet A. 2015 Mar;167A(3):621-8. doi: 10.1002/ajmg.a.36939. [PubMed:25691415  ] 
Associated OMIM IDs
  • 302060   (3-methylglutaconic aciduria type II, X-linked)
    • 

  • 114500   (Colorectal cancer)
    • 

  • 137215   (Stomach cancer)
    • 

  • 181500   (Schizophrenia)
    • 

  • 203740   (2-Ketoglutarate dehydrogenase complex deficiency)
    • 

  • 246450   (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
    • 

  • 605911   (3-Hydroxy-3-Methylglutaryl-CoA Synthase Deficiency)
    • 

  • 256000   (Leigh's syndrome, subacute necrotizing encephalopathy, SNE)
    • 

  • 255125   (Myopathy with lactic acidosis, hereditary)
    • 

  • 229700   (Fructose-1,6-diphosphatase deficiency)
    • 

  • 614741   (Mitochondrial pyruvate carrier deficiency)
    • 

  • 312170   (Pyruvate dehydrogenase deficiency)
    • 

  • 608782   (Pyruvate dehydrogenase phosphatase deficiency)
    • 

  • 246900   (Dihydrolipoamide Dehydrogenase Deficiency)
    • 

  • 616299   (Lipoyltransferase 1 Deficiency)
    • 

  • 603358   (GRACILE syndrome)
    • 

  • 210200   (3-Methyl-crotonyl-glycinuria)
    • 

  • 606812   (Fumarase deficiency)
    • 

  • 104300   (Alzheimer's disease)
    • 

  • 600274   (Frontotemporal dementia)
    • 

  • 222100   (Diabetes mellitus type 1)
    • 

  • 610247   (Eosinophilic esophagitis)
    • 

  • 557000   (Pearson Syndrome)
DrugBank IDDB00119  
Phenol Explorer Compound IDNot Available
FooDB IDFDB031141  
KNApSAcK IDC00001200  
Chemspider ID1031  
KEGG Compound IDC00022  
BioCyc IDPYRUVATE  
BiGG IDNot Available
Wikipedia LinkPyruvic_acid  
METLIN IDNot Available
PubChem Compound1060  
PDB IDNot Available
ChEBI ID32816  
Food Biomarker OntologyNot Available
VMH IDPYR  
MarkerDB IDMDB00000118  
References
Synthesis ReferenceXiang, Wei; Okita, Motomu.  Preparation of pyruvic acid.    Jpn. Kokai Tokkyo Koho  (2003),     5 pp.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Nielsen J, Ytrebo LM, Borud O: Lactate and pyruvate concentrations in capillary blood from newborns. Acta Paediatr. 1994 Sep;83(9):920-2. [PubMed:7819686  ] 
  2. Ka T, Yamamoto T, Moriwaki Y, Kaya M, Tsujita J, Takahashi S, Tsutsumi Z, Fukuchi M, Hada T: Effect of exercise and beer on the plasma concentration and urinary excretion of purine bases. J Rheumatol. 2003 May;30(5):1036-42. [PubMed:12734903  ] 
  3. Talseth T, Haegele KD, McNay JL, Skrdlant HB, Clementi WA, Shepherd AM: Pharmacokinetics and cardiovascular effects in rabbits of a major hydralazine metabolite, the hydralazine pyruvic-acid hydrazone. J Pharmacol Exp Ther. 1979 Dec;211(3):509-13. [PubMed:512915  ] 
  4. Subramanian A, Gupta A, Saxena S, Gupta A, Kumar R, Nigam A, Kumar R, Mandal SK, Roy R: Proton MR CSF analysis and a new software as predictors for the differentiation of meningitis in children. NMR Biomed. 2005 Jun;18(4):213-25. [PubMed:15627241  ] 
  5. Zupke C, Sinskey AJ, Stephanopoulos G: Intracellular flux analysis applied to the effect of dissolved oxygen on hybridomas. Appl Microbiol Biotechnol. 1995 Dec;44(1-2):27-36. [PubMed:8579834  ] 
  6. Reece PA, Cozamanis I, Zacest R: Selective high-performance liquid chromatographic assays for hydralazine and its metabolites in plasma of man. J Chromatogr. 1980 Mar 14;181(3-4):427-40. [PubMed:7391156  ] 
  7. Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed:8087979  ] 
  8. Meijer-Severs GJ, Van Santen E, Meijer BC: Short-chain fatty acid and organic acid concentrations in feces of healthy human volunteers and their correlations with anaerobe cultural counts during systemic ceftriaxone administration. Scand J Gastroenterol. 1990 Jul;25(7):698-704. [PubMed:2396083  ] 
  9. Silwood CJ, Lynch E, Claxson AW, Grootveld MC: 1H and (13)C NMR spectroscopic analysis of human saliva. J Dent Res. 2002 Jun;81(6):422-7. [PubMed:12097436  ] 
  10. Nakayama Y, Kinoshita A, Tomita M: Dynamic simulation of red blood cell metabolism and its application to the analysis of a pathological condition. Theor Biol Med Model. 2005 May 9;2:18. [PubMed:15882454  ] 
  11. Elling D, Bader K: [Biochemical changes in cervix mucus in stepwise malignant transformation of cervix epithelium]. Zentralbl Gynakol. 1990;112(9):555-60. [PubMed:2378186  ] 
  12. Mongan PD, Capacchione J, West S, Karaian J, Dubois D, Keneally R, Sharma P: Pyruvate improves redox status and decreases indicators of hepatic apoptosis during hemorrhagic shock in swine. Am J Physiol Heart Circ Physiol. 2002 Oct;283(4):H1634-44. Epub 2002 Jun 20. [PubMed:12234818  ] 
  13. Hoffmann GF, Meier-Augenstein W, Stockler S, Surtees R, Rating D, Nyhan WL: Physiology and pathophysiology of organic acids in cerebrospinal fluid. J Inherit Metab Dis. 1993;16(4):648-69. [PubMed:8412012  ] 
  14. Foster KJ, Alberti KG, Hinks L, Lloyd B, Postle A, Smythe P, Turnell DC, Walton R: Blood intermediary metabolite and insulin concentrations after an overnight fast: reference ranges for adults, and interrelations. Clin Chem. 1978 Sep;24(9):1568-72. [PubMed:688619  ] 
  15. Tsuchiya H, Hashizume I, Tokunaga T, Tatsumi M, Takagi N, Hayashi T: High-performance liquid chromatography of alpha-keto acids in human saliva. Arch Oral Biol. 1983;28(11):989-92. [PubMed:6581765  ] 
  16. Wendisch VF: Microbial Production of Amino Acid-Related Compounds. Adv Biochem Eng Biotechnol. 2017;159:255-269. doi: 10.1007/10_2016_34. [PubMed:27872963  ] 
  17. Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212  ] 
  Only showing the first  10 proteins. There  are 59 proteins in total. 
Show all enzymes and transporters
Enzymes
  Enzyme Details
1. Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
General function:
Involved in catalytic activity
Specific function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Gene Name:
PDHB
Uniprot ID:
P11177  
Molecular weight:
39233.1
Reactions
Pyruvic acid + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine → [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2)details
Pyruvic acid + Thiamine pyrophosphate → 2-(a-Hydroxyethyl)thiamine diphosphate + Carbon dioxidedetails
Pyruvic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + Carbon dioxidedetails
References
  1. Kumar V, Rangaraj N, Shivaji S: Activity of pyruvate dehydrogenase A (PDHA) in hamster spermatozoa correlates positively with hyperactivation and is associated with sperm capacitation. Biol Reprod. 2006 Nov;75(5):767-77. Epub 2006 Jul 19. [PubMed:16855207  ] 
  Enzyme Details
2. Pyruvate carboxylase, mitochondrial
General function:
Involved in catalytic activity
Specific function:
Pyruvate carboxylase catalyzes a 2-step reaction, involving the ATP-dependent carboxylation of the covalently attached biotin in the first step and the transfer of the carboxyl group to pyruvate in the second. Catalyzes in a tissue specific manner, the initial reactions of glucose (liver, kidney) and lipid (adipose tissue, liver, brain) synthesis from pyruvate.

Gene Name:
PC
Uniprot ID:
P11498  
Molecular weight:
129632.565
Reactions
Adenosine triphosphate + Pyruvic acid + Hydrogen carbonate → ADP + Phosphate + Oxalacetic aciddetails
References
  1. Jitrapakdee S, Vidal-Puig A, Wallace JC: Anaplerotic roles of pyruvate carboxylase in mammalian tissues. Cell Mol Life Sci. 2006 Apr;63(7-8):843-54. [PubMed:16505973  ] 
  2. Simpson NE, Khokhlova N, Oca-Cossio JA, Constantinidis I: Insights into the role of anaplerosis in insulin secretion: A 13C NMR study. Diabetologia. 2006 Jun;49(6):1338-48. Epub 2006 Mar 31. [PubMed:16575559  ] 
  3. Jensen MV, Joseph JW, Ilkayeva O, Burgess S, Lu D, Ronnebaum SM, Odegaard M, Becker TC, Sherry AD, Newgard CB: Compensatory responses to pyruvate carboxylase suppression in islet beta-cells. Preservation of glucose-stimulated insulin secretion. J Biol Chem. 2006 Aug 4;281(31):22342-51. Epub 2006 Jun 1. [PubMed:16740637  ] 
  4. Ikeda K, Yukihiro Hiraoka B, Iwai H, Matsumoto T, Mineki R, Taka H, Takamori K, Ogawa H, Yamakura F: Detection of 6-nitrotryptophan in proteins by Western blot analysis and its application for peroxynitrite-treated PC12 cells. Nitric Oxide. 2007 Feb;16(1):18-28. Epub 2006 May 4. [PubMed:16765071  ] 
  5. Liu L, Li Y, Zhu Y, Du G, Chen J: Redistribution of carbon flux in Torulopsis glabrata by altering vitamin and calcium level. Metab Eng. 2007 Jan;9(1):21-9. Epub 2006 Aug 12. [PubMed:17008113  ] 
  Enzyme Details
3. Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Gene Name:
PDHA1
Uniprot ID:
P08559  
Molecular weight:
43295.255
Reactions
Pyruvic acid + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine → [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2)details
Pyruvic acid + Thiamine pyrophosphate → 2-(a-Hydroxyethyl)thiamine diphosphate + Carbon dioxidedetails
Pyruvic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + Carbon dioxidedetails
Coenzyme A + NAD + Pyruvic acid → Carbon dioxide + Hydrogen Ion + NADH + Acetyl-CoAdetails
  Enzyme Details
4. Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Gene Name:
PDHA2
Uniprot ID:
P29803  
Molecular weight:
42932.855
Reactions
Pyruvic acid + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine → [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO(2)details
Pyruvic acid + Thiamine pyrophosphate → 2-(a-Hydroxyethyl)thiamine diphosphate + Carbon dioxidedetails
Pyruvic acid + Enzyme N6-(lipoyl)lysine → [Dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + Carbon dioxidedetails
  Enzyme Details
5. Dihydrolipoyl dehydrogenase, mitochondrial
General function:
Involved in oxidoreductase activity
Specific function:
Lipoamide dehydrogenase is a component of the glycine cleavage system as well as of the alpha-ketoacid dehydrogenase complexes. Involved in the hyperactivation of spermatazoa during capacitation and in the spermatazoal acrosome reaction.

Gene Name:
DLD
Uniprot ID:
P09622  
Molecular weight:
54176.91
Reactions
Coenzyme A + NAD + Pyruvic acid → Carbon dioxide + Hydrogen Ion + NADH + Acetyl-CoAdetails
  Enzyme Details
6. Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
General function:
Involved in acyltransferase activity
Specific function:
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.

Gene Name:
DLAT
Uniprot ID:
P10515  
Molecular weight:
68996.03
Reactions
Coenzyme A + NAD + Pyruvic acid → Carbon dioxide + Hydrogen Ion + NADH + Acetyl-CoAdetails
  Enzyme Details
7. D-amino-acid oxidase
General function:
Involved in D-amino-acid oxidase activity
Specific function:
Regulates the level of the neuromodulator D-serine in the brain. Has high activity towards D-DOPA and contributes to dopamine synthesis. Could act as a detoxifying agent which removes D-amino acids accumulated during aging. Acts on a variety of D-amino acids with a preference for those having small hydrophobic side chains followed by those bearing polar, aromatic, and basic groups. Does not act on acidic amino acids.

Gene Name:
DAO
Uniprot ID:
P14920  
Molecular weight:
39473.75
  Enzyme Details
8. 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
References
  1. Andersen G, Andersen B, Dobritzsch D, Schnackerz KD, Piskur J: A gene duplication led to specialized gamma-aminobutyrate and beta-alanine aminotransferase in yeast. FEBS J. 2007 Apr;274(7):1804-17. Epub 2007 Mar 12. [PubMed:17355287  ] 
  2. Schmidt C, Hofmann U, Kohlmuller D, Murdter T, Zanger UM, Schwab M, Hoffmann GF: Comprehensive analysis of pyrimidine metabolism in 450 children with unspecific neurological symptoms using high-pressure liquid chromatography-electrospray ionization tandem mass spectrometry. J Inherit Metab Dis. 2005;28(6):1109-22. [PubMed:16435204  ] 
  3. Chen X, Ji ZL, Chen YZ: TTD: Therapeutic Target Database. Nucleic Acids Res. 2002 Jan 1;30(1):412-5. [PubMed:11752352  ] 
  Enzyme Details
9. Alanine--glyoxylate aminotransferase 2, mitochondrial
General function:
Involved in transaminase activity
Specific function:
Can metabolize asymmetric dimethylarginine (ADMA) via transamination to alpha-keto-delta-(NN-dimethylguanidino) valeric acid (DMGV). ADMA is a potent inhibitor of nitric-oxide (NO) synthase, and this activity provides mechanism through which the kidney regulates blood pressure.

Gene Name:
AGXT2
Uniprot ID:
Q9BYV1  
Molecular weight:
57155.905
Reactions
L-Alanine + Glyoxylic acid → Pyruvic acid + Glycinedetails
(R)-beta-Aminoisobutyric acid + Pyruvic acid → 2-Methyl-3-oxopropanoic acid + L-Alaninedetails
References
  1. Overington JP, Al-Lazikani B, Hopkins AL: How many drug targets are there? Nat Rev Drug Discov. 2006 Dec;5(12):993-6. [PubMed:17139284  ] 
  2. Imming P, Sinning C, Meyer A: Drugs, their targets and the nature and number of drug targets. Nat Rev Drug Discov. 2006 Oct;5(10):821-34. [PubMed:17016423  ] 
  3. Tamaki N, Fujimoto S, Mizota C, Kaneko M, Kikugawa M: Inhibitory effect of 6-azauracil on beta-alanine metabolism in rat. J Nutr Sci Vitaminol (Tokyo). 1989 Oct;35(5):451-61. [PubMed:2632679  ] 
  Enzyme Details
10. S-adenosylmethionine decarboxylase proenzyme
General function:
Involved in adenosylmethionine decarboxylase activity
Specific function:
Not Available
Gene Name:
AMD1
Uniprot ID:
P17707  
Molecular weight:
21301.015
Transporters
  Enzyme Details
1. Monocarboxylate transporter 4
General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate
Gene Name:
SLC16A3
Uniprot ID:
O15427  
Molecular weight:
49468.9
References
  1. Shimada A, Nakagawa Y, Morishige H, Yamamoto A, Fujita T: Functional characteristics of H+ -dependent nicotinate transport in primary cultures of astrocytes from rat cerebral cortex. Neurosci Lett. 2006 Jan 16;392(3):207-12. Epub 2005 Oct 5. [PubMed:16213084  ] 
  2. Manning Fox JE, Meredith D, Halestrap AP: Characterisation of human monocarboxylate transporter 4 substantiates its role in lactic acid efflux from skeletal muscle. J Physiol. 2000 Dec 1;529 Pt 2:285-93. [PubMed:11101640  ] 
  Enzyme Details
2. Monocarboxylate transporter 1
General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate
Gene Name:
SLC16A1
Uniprot ID:
P53985  
Molecular weight:
53957.7
References
  1. Duerr JM, Tucker K: Pyruvate transport in isolated cardiac mitochondria from two species of amphibian exhibiting dissimilar aerobic scope: Bufo marinus and Rana catesbeiana. J Exp Zool A Ecol Genet Physiol. 2007 Aug 1;307(8):425-38. [PubMed:17583564  ] 
  2. Han M, Trotta P, Coleman C, Linask KK: MCT-4, A511/Basigin and EF5 expression patterns during early chick cardiomyogenesis indicate cardiac cell differentiation occurs in a hypoxic environment. Dev Dyn. 2006 Jan;235(1):124-31. [PubMed:16110503  ] 
  3. Shimoyama Y, Akihara Y, Kirat D, Iwano H, Hirayama K, Kagawa Y, Ohmachi T, Matsuda K, Okamoto M, Kadosawa T, Yokota H, Taniyama H: Expression of monocarboxylate transporter 1 in oral and ocular canine melanocytic tumors. Vet Pathol. 2007 Jul;44(4):449-57. [PubMed:17606506  ] 
  4. Shimada A, Nakagawa Y, Morishige H, Yamamoto A, Fujita T: Functional characteristics of H+ -dependent nicotinate transport in primary cultures of astrocytes from rat cerebral cortex. Neurosci Lett. 2006 Jan 16;392(3):207-12. Epub 2005 Oct 5. [PubMed:16213084  ] 
  5. Philp A, Macdonald AL, Watt PW: Lactate--a signal coordinating cell and systemic function. J Exp Biol. 2005 Dec;208(Pt 24):4561-75. [PubMed:16326938  ] 
  6. Broer S, Rahman B, Pellegri G, Pellerin L, Martin JL, Verleysdonk S, Hamprecht B, Magistretti PJ: Comparison of lactate transport in astroglial cells and monocarboxylate transporter 1 (MCT 1) expressing Xenopus laevis oocytes. Expression of two different monocarboxylate transporters in astroglial cells and neurons. J Biol Chem. 1997 Nov 28;272(48):30096-102. [PubMed:9374487  ] 
  7. Broer S, Schneider HP, Broer A, Rahman B, Hamprecht B, Deitmer JW: Characterization of the monocarboxylate transporter 1 expressed in Xenopus laevis oocytes by changes in cytosolic pH. Biochem J. 1998 Jul 1;333 ( Pt 1):167-74. [PubMed:9639576  ] 
  8. Lin RY, Vera JC, Chaganti RS, Golde DW: Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter. J Biol Chem. 1998 Oct 30;273(44):28959-65. [PubMed:9786900  ] 
  Enzyme Details
3. Monocarboxylate transporter 8
General function:
Involved in transmembrane transport
Specific function:
Very active and specific thyroid hormone transporter. Stimulates cellular uptake of thyroxine (T4), triiodothyronine (T3), reverse triiodothyronine (rT3) and diidothyronine. Does not transport Leu, Phe, Trp or Tyr
Gene Name:
SLC16A2
Uniprot ID:
P36021  
Molecular weight:
59510.9
References
  1. Bonen A, Heynen M, Hatta H: Distribution of monocarboxylate transporters MCT1-MCT8 in rat tissues and human skeletal muscle. Appl Physiol Nutr Metab. 2006 Feb;31(1):31-9. [PubMed:16604139  ] 
  Enzyme Details
4. Monocarboxylate transporter 2
General function:
Involved in transmembrane transport
Specific function:
Proton-linked monocarboxylate transporter. Catalyzes the rapid transport across the plasma membrane of many monocarboxylates such as lactate, pyruvate, branched-chain oxo acids derived from leucine, valine and isoleucine, and the ketone bodies acetoacetate, beta-hydroxybutyrate and acetate. MCT2 is a high affinity pyruvate transporter
Gene Name:
SLC16A7
Uniprot ID:
O60669  
Molecular weight:
52185.7
References
  1. Bonen A, Heynen M, Hatta H: Distribution of monocarboxylate transporters MCT1-MCT8 in rat tissues and human skeletal muscle. Appl Physiol Nutr Metab. 2006 Feb;31(1):31-9. [PubMed:16604139  ] 
  2. Hinoi E, Takarada T, Tsuchihashi Y, Fujimori S, Moriguchi N, Wang L, Uno K, Yoneda Y: A molecular mechanism of pyruvate protection against cytotoxicity of reactive oxygen species in osteoblasts. Mol Pharmacol. 2006 Sep;70(3):925-35. Epub 2006 Jun 9. [PubMed:16766717  ] 
  3. Yoshida Y, Holloway GP, Ljubicic V, Hatta H, Spriet LL, Hood DA, Bonen A: Negligible direct lactate oxidation in subsarcolemmal and intermyofibrillar mitochondria obtained from red and white rat skeletal muscle. J Physiol. 2007 Aug 1;582(Pt 3):1317-35. Epub 2007 Jun 7. [PubMed:17556391  ] 
  4. de Laplanche E, Gouget K, Cleris G, Dragounoff F, Demont J, Morales A, Bezin L, Godinot C, Perriere G, Mouchiroud D, Simonnet H: Physiological oxygenation status is required for fully differentiated phenotype in kidney cortex proximal tubules. Am J Physiol Renal Physiol. 2006 Oct;291(4):F750-60. Epub 2006 Apr 4. [PubMed:16597615  ] 
  5. Pierre K, Pellerin L: Monocarboxylate transporters in the central nervous system: distribution, regulation and function. J Neurochem. 2005 Jul;94(1):1-14. [PubMed:15953344  ] 
  6. Broer S, Broer A, Schneider HP, Stegen C, Halestrap AP, Deitmer JW: Characterization of the high-affinity monocarboxylate transporter MCT2 in Xenopus laevis oocytes. Biochem J. 1999 Aug 1;341 ( Pt 3):529-35. [PubMed:10417314  ] 
  7. Lin RY, Vera JC, Chaganti RS, Golde DW: Human monocarboxylate transporter 2 (MCT2) is a high affinity pyruvate transporter. J Biol Chem. 1998 Oct 30;273(44):28959-65. [PubMed:9786900  ] 
  Enzyme Details
5. Monocarboxylate transporter 10
General function:
Involved in transmembrane transport
Specific function:
Sodium-independent transporter that mediates the update of aromatic acid. Can function as a net efflux pathway for aromatic amino acids in the basosolateral epithelial cells
Gene Name:
SLC16A10
Uniprot ID:
Q8TF71  
Molecular weight:
55492.1
References
  1. Kim DK, Kanai Y, Chairoungdua A, Matsuo H, Cha SH, Endou H: Expression cloning of a Na+-independent aromatic amino acid transporter with structural similarity to H+/monocarboxylate transporters. J Biol Chem. 2001 May 18;276(20):17221-8. Epub 2001 Feb 20. [PubMed:11278508  ] 
  2. Kim DK, Kanai Y, Matsuo H, Kim JY, Chairoungdua A, Kobayashi Y, Enomoto A, Cha SH, Goya T, Endou H: The human T-type amino acid transporter-1: characterization, gene organization, and chromosomal location. Genomics. 2002 Jan;79(1):95-103. [PubMed:11827462  ] 
  Enzyme Details
6. Solute carrier organic anion transporter family member 2A1
General function:
Not Available
Specific function:
May mediate the release of newly synthesized prostaglandins from cells, the transepithelial transport of prostaglandins, and the clearance of prostaglandins from the circulation. Transports PGD2, as well as PGE1, PGE2 and PGF2A
Gene Name:
SLCO2A1
Uniprot ID:
Q92959  
Molecular weight:
70117.0
References
  1. Chan BS, Endo S, Kanai N, Schuster VL: Identification of lactate as a driving force for prostanoid transport by prostaglandin transporter PGT. Am J Physiol Renal Physiol. 2002 Jun;282(6):F1097-102. [PubMed:11997326  ] 
  Only showing the first  10 proteins. There  are 59 proteins in total. 
Show all enzymes and transporters