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Human Metabolome Database Version 3.5

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Showing metabocard for 2-Ketobutyric acid (HMDB00005)

Record Information
Version 3.5
Creation Date 2005-11-16 08:48:42 -0700
Update Date 2013-02-08 17:07:44 -0700
HMDB ID HMDB00005
Secondary Accession Numbers
  • HMDB06544
Metabolite Identification
Common Name 2-Ketobutyric acid
Description 2-Ketobutyric acid is a substance that is involved in the metabolism of many amino acids (glycine, methionine, valine, leucine, serine, threonine, isoleucine) as well as propanoate metabolism and C-5 branched dibasic acid metabolism. More specifically, alpha-ketobutyric acid is a product of the lysis of cystathionine. It is also one of the degradation products of threonine. It can be converted to propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted to succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle.
Structure Thumb
Download: MOL | SDF | SMILES | InChI
Display: 2D Structure | 3D Structure
Synonyms
  1. 2-Ketobutanoate
  2. 2-Ketobutanoic acid
  3. 2-Ketobutyrate
  4. 2-Oxo-Butanoate
  5. 2-Oxo-Butanoic acid
  6. 2-Oxo-Butyrate
  7. 2-Oxo-Butyric acid
  8. 2-Oxo-N-butyrate
  9. 2-Oxo-N-butyric acid
  10. 2-Oxobutanoate
  11. 2-Oxobutanoic acid
  12. 2-Oxobutyrate
  13. 2-Oxobutyric acid
  14. 3-Methylpyruvate
  15. 3-Methylpyruvic acid
  16. a-Keto-N-Butyrate
  17. a-Keto-N-Butyric acid
  18. a-Ketobutyrate
  19. a-Ketobutyric acid
  20. a-Oxo-N-butyrate
  21. a-Oxo-N-butyric acid
  22. a-Oxobutyrate
  23. a-Oxobutyric acid
  24. alpha-Keto-N-butyrate
  25. alpha-Keto-N-butyric acid
  26. alpha-Ketobutric acid
  27. alpha-Ketobutyrate
  28. alpha-Ketobutyric acid
  29. alpha-Oxo-N-butyrate
  30. alpha-Oxo-N-butyric acid
  31. alpha-Oxobutyrate
  32. alpha-Oxobutyric acid
  33. Methyl-Pyruvate
  34. Methyl-Pyruvic acid
  35. Propionyl-formate
  36. Propionyl-formic acid
Chemical Formula C4H6O3
Average Molecular Weight 102.0886
Monoisotopic Molecular Weight 102.031694058
IUPAC Name 2-oxobutanoic acid
Traditional IUPAC Name 2-oxobutanoic acid
CAS Registry Number 600-18-0
SMILES CCC(=O)C(O)=O
InChI Identifier InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)
InChI Key TYEYBOSBBBHJIV-UHFFFAOYSA-N
Chemical Taxonomy
Kingdom Organic Compounds
Super Class Organic Acids and Derivatives
Class Keto-Acids and Derivatives
Sub Class Short-chain Keto Acids and Derivatives
Other Descriptors
  • 2-oxo monocarboxylic acid(ChEBI)
  • Aliphatic Acyclic Compounds
  • Alpha Keto-Acids and Derivatives
  • Organic Compounds
  • Oxo fatty acids(KEGG)
  • Oxo fatty acids(Lipidmaps)
  • Straight Chain Fatty Acids
  • short-chain fatty acid(ChEBI)
  • straight-chain saturated fatty acid(ChEBI)
Substituents
  • Carboxylic Acid
  • Ketone
Direct Parent Short-chain Keto Acids and Derivatives
Ontology
Status Detected and Quantified
Origin
  • Endogenous
Biofunction
  • Component of Cysteine metabolism
  • Component of Glycine, serine and threonine metabolism
  • Component of Methionine metabolism
  • Component of Nitrogen metabolism
  • Component of Selenoamino acid metabolism
Application Not Available
Cellular locations
  • Cytoplasm
Physical Properties
State Solid
Experimental Properties
Property Value Reference
Melting Point 33 °C Not Available
Boiling Point Not Available Not Available
Water Solubility 119 mg/mL Not Available
LogP Not Available Not Available
Predicted Properties
Property Value Source
Water Solubility 79.2 g/L ALOGPS
LogP 0.07 ALOGPS
LogP 0.77 ChemAxon
LogS -0.11 ALOGPS
pKa (strongest acidic) 3.19 ChemAxon
pKa (strongest basic) -9.7 ChemAxon
Hydrogen Acceptor Count 3 ChemAxon
Hydrogen Donor Count 1 ChemAxon
Polar Surface Area 54.37 A2 ChemAxon
Rotatable Bond Count 2 ChemAxon
Refractivity 22.62 ChemAxon
Polarizability 9.21 ChemAxon
Formal Charge 0 ChemAxon
Physiological Charge -1 ChemAxon
Spectra
1H NMR Spectrum
MS/MS Spectrum Quattro_QQQ 10
MS/MS Spectrum Quattro_QQQ 25
MS/MS Spectrum Quattro_QQQ 40
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies )
MS/MS Spectrum LC-ESI-QTOF (UPLC Q-Tof Premier, Waters)
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
MS/MS Spectrum GC-MS
[1H,13C] 2D NMR Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Urine
Tissue Location Not Available
Pathways
Name SMPDB Link KEGG Link
Methionine Metabolism SMP00033 map00270 Link_out
Glycine and Serine Metabolism SMP00004 map00260 Link_out
Threonine and 2-Oxobutanoate Degradation SMP00452 Not Available
Homocysteine Degradation SMP00455 Not Available
Normal Concentrations
Biofluid Status Value Age Sex Condition Comments
Blood Detected and Quantified
Article_icon
3.33 +/- 5.00 uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
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9.4 (3.4-15.4) uM Adult (>18 years old) Both Normal Not Available
Blood Detected and Quantified
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8.6 (6.0-11.2) uM Children (1-13 year old) Both Normal Not Available
Cerebrospinal Fluid (CSF) Detected and Quantified
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4.8 (3.4-6.2) uM Children (1-13 year old) Both Normal Not Available
Urine Detected and Quantified
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0.39 (0.05-2.2) umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Urine Detected and Quantified
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0.13 (0.04-0.51) umol/mmol creatinine Adult (>18 years old) Female Normal Not Available
Urine Detected and not Quantified
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Not Applicable Adult (>18 years old) Both Normal Urine compound detected by GC-MS
Urine Detected and Quantified
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2.9 (0.7-5.4) umol/mmol creatinine Adult (>18 years old) Both Normal urine by NMR
Urine Detected and Quantified
Article_icon
2.6 (1.1-4.1) umol/mmol creatinine Adult (>18 years old) Male Normal Not Available
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease References None
Associated OMIM IDs None
DrugBank ID DB04553 Link_out
Phenol Explorer Compound ID Not Available
Phenol Explorer Metabolite ID Not Available
FoodDB ID FDB003359
KNApSAcK ID C00019675 Link_out
Chemspider ID 57 Link_out
KEGG Compound ID C00109 Link_out
BioCyc ID 2-OXOBUTANOATE Link_out
BiGG ID 33889 Link_out
Wikipedia Link Alpha-ketobutyric_acid Link_out
NuGOwiki Link HMDB00005 Link_out
Metagene Link HMDB00005 Link_out
METLIN ID Not Available
PubChem Compound 58 Link_out
PDB ID 2KT Link_out
ChEBI ID 30831 Link_out
References
Synthesis Reference Figge, Rainer; Lux, Fabien; Raynaud, Celine; Soucaille, Philippe. Production of a-ketobutyrate by engineered Escherichia coli.PCT Int. Appl. (2006), 31pp.
Material Safety Data Sheet (MSDS) Not Available
General References
  1. Sprague CL, Elfarra AA: Detection of carboxylic acids and inhibition of hippuric acid formation in rats treated with 3-butene-1,2-diol, a major metabolite of 1,3-butadiene. Drug Metab Dispos. 2003 Aug;31(8):986-92. Pubmed: 12867486 Link_out
  2. Yaegaki K, Sanada K: Biochemical and clinical factors influencing oral malodor in periodontal patients. J Periodontol. 1992 Sep;63(9):783-9. Pubmed: 1474480 Link_out
  3. Yaegaki K, Sanada K: Effects of a two-phase oil-water mouthwash on halitosis. Clin Prev Dent. 1992 Jan-Feb;14(1):5-9. Pubmed: 1499240 Link_out
  4. Yang W, Roth KS: Defect in alpha-ketobutyrate metabolism: a new inborn error. Clin Chim Acta. 1985 Jan 30;145(2):173-82. Pubmed: 3918815 Link_out
Enzymes
Name: Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
Reactions:
  • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
Gene Name: PDHB
Uniprot ID: P11177 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial
Reactions:
  • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
Gene Name: PDHA1
Uniprot ID: P08559 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Pyruvate dehydrogenase E1 component subunit alpha, testis-specific form, mitochondrial
Reactions:
  • pyruvate + [dihydrolipoyllysine-residue acetyltransferase] lipoyllysine = [dihydrolipoyllysine-residue acetyltransferase] S-acetyldihydrolipoyllysine + CO2 [RN:R01699]
Gene Name: PDHA2
Uniprot ID: P29803 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
Reactions:
  • 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
Gene Name: BCKDHB
Uniprot ID: P21953 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
Reactions:
  • 3-methyl-2-oxobutanoate + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine = [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO2 [RN:R01701]
Gene Name: BCKDHA
Uniprot ID: P12694 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: 2-oxoglutarate dehydrogenase, mitochondrial
Reactions:
  • 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine = [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2 [RN:R01700]
Gene Name: OGDH
Uniprot ID: Q02218 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Cystathionine gamma-lyase
Reactions:
  • (1) L-cystathionine + H2O = L-cysteine + NH3 + 2-oxobutanoate (overall reaction) [RN:R01001]
  • (2) (1a) L-cystathionine = L-cysteine + 2-ammoniobut-2-enoate [RN:R08632]
  • (3) (1b) 2-ammoniobut-2-enoate + H2O = 2-oxobutanoate + NH3 (spontaneous) [RN:R08637]
Gene Name: CTH
Uniprot ID: P32929 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: L-lactate dehydrogenase A-like 6A
Reactions:
  • (S)-lactate + NAD+ = pyruvate + NADH + H+ [RN:R00703]
Gene Name: LDHAL6A
Uniprot ID: Q6ZMR3 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Urocanate hydratase
Reactions:
  • 3-(5-oxo-4,5-dihydro-3H-imidazol-4-yl)propanoate = urocanate + H2O [RN:R02914]
Gene Name: UROC1
Uniprot ID: Q96N76 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial
Reactions:
  • ATP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] = ADP + [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] phosphate
Gene Name: BCKDK
Uniprot ID: O14874 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Trimethyllysine dioxygenase, mitochondrial
Reactions:
  • N6,N6,N6-trimethyl-L-lysine + 2-oxoglutarate + O2 = 3-hydroxy-N6,N6,N6-trimethyl-L-lysine + succinate + CO2 [RN:R03451]
Gene Name: TMLHE
Uniprot ID: Q9NVH6 Link_out
Protein Sequence: FASTA
Gene Sequence: FASTA
Name: Alpha-ketoglutarate-dependent dioxygenase alkB homolog 2
Reactions:
    Gene Name: ALKBH2
    Uniprot ID: Q6NS38 Link_out
    Protein Sequence: FASTA
    Gene Sequence: FASTA
    Name: Alpha-ketoglutarate-dependent dioxygenase alkB homolog 3
    Reactions:
      Gene Name: ALKBH3
      Uniprot ID: Q96Q83 Link_out
      Protein Sequence: FASTA
      Gene Sequence: FASTA
      Name: 2-oxoglutarate dehydrogenase-like, mitochondrial
      Reactions:
        Gene Name: OGDHL
        Uniprot ID: Q9ULD0 Link_out
        Protein Sequence: FASTA
        Gene Sequence: FASTA
        Name: 2-oxoglutarate receptor 1
        Reactions:
          Gene Name: OXGR1
          Uniprot ID: Q96P68 Link_out
          Protein Sequence: FASTA
          Gene Sequence: FASTA