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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2014-10-29 21:49:57 UTC
HMDB IDHMDB00005
Secondary Accession Numbers
  • HMDB06544
Metabolite Identification
Common Name2-Ketobutyric acid
Description2-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
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 FormulaC4H6O3
Average Molecular Weight102.0886
Monoisotopic Molecular Weight102.031694058
IUPAC Name2-oxobutanoic acid
Traditional Name2-oxobutanoic acid
CAS Registry Number600-18-0
SMILES
CCC(=O)C(O)=O
InChI Identifier
InChI=1S/C4H6O3/c1-2-3(5)4(6)7/h2H2,1H3,(H,6,7)
InChI KeyTYEYBOSBBBHJIV-UHFFFAOYSA-N
Chemical Taxonomy
KingdomOrganic Compounds
Super ClassOrganic Acids and Derivatives
ClassKeto-Acids and Derivatives
Sub ClassShort-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 ParentShort-chain Keto Acids and Derivatives
Ontology
StatusDetected 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
ApplicationNot Available
Cellular locations
  • Cytoplasm
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point33 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility119 mg/mLNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility79.2 g/LALOGPS
logP0.07ALOGPS
logP0.77ChemAxon
logS-0.11ALOGPS
pKa (Strongest Acidic)3.19ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity22.62ChemAxon
Polarizability9.21ChemAxon
Spectra
SpectraGC-MSMS/MSLC-MS1D NMR2D NMR
Biological Properties
Cellular Locations
  • Cytoplasm
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationNot Available
Pathways
NameSMPDB LinkKEGG Link
Glycine and Serine MetabolismSMP00004map00260
Homocysteine DegradationSMP00455Not Available
Methionine MetabolismSMP00033map00270
Threonine and 2-Oxobutanoate DegradationSMP00452Not Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified3.33 +/- 5.00 uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified9.4 (3.4-15.4) uMAdult (>18 years old)BothNormal details
BloodDetected and Quantified8.6 (6.0-11.2) uMChildren (1-13 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified4.8 (3.4-6.2) uMChildren (1-13 years old)BothNormal details
SalivaDetected and Quantified0.0412 +/- 0.214 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified2.62 +/- 4.13 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified4.06 +/- 8.39 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified4.12 +/- 4.41 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified0.39 (0.05-2.2) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.13 (0.04-0.51) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected but not QuantifiedNot ApplicableAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.9 (0.7-5.4) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified2.6 (1.1-4.1) umol/mmol creatinineAdult (>18 years old)MaleNormal
    • Shaykhutdinov RA,...
details
Abnormal Concentrations
Not Available
Associated Disorders and Diseases
Disease ReferencesNone
Associated OMIM IDsNone
DrugBank IDDB04553
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB003359
KNApSAcK IDC00019675
Chemspider ID57
KEGG Compound IDC00109
BioCyc ID2-OXOBUTANOATE
BiGG ID33889
Wikipedia LinkAlpha-ketobutyric_acid
NuGOwiki LinkHMDB00005
Metagene LinkHMDB00005
METLIN IDNot Available
PubChem Compound58
PDB ID2KT
ChEBI ID30831
References
Synthesis ReferenceFigge, 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
  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
  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
  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

Enzymes

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
References
  1. Paxton R, Scislowski PW, Davis EJ, Harris RA: Role of branched-chain 2-oxo acid dehydrogenase and pyruvate dehydrogenase in 2-oxobutyrate metabolism. Biochem J. 1986 Mar 1;234(2):295-303. Pubmed: 3718468
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
References
  1. Paxton R, Scislowski PW, Davis EJ, Harris RA: Role of branched-chain 2-oxo acid dehydrogenase and pyruvate dehydrogenase in 2-oxobutyrate metabolism. Biochem J. 1986 Mar 1;234(2):295-303. Pubmed: 3718468
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
References
  1. Paxton R, Scislowski PW, Davis EJ, Harris RA: Role of branched-chain 2-oxo acid dehydrogenase and pyruvate dehydrogenase in 2-oxobutyrate metabolism. Biochem J. 1986 Mar 1;234(2):295-303. Pubmed: 3718468
General function:
Involved in catalytic activity
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHB
Uniprot ID:
P21953
Molecular weight:
43122.065
General function:
Involved in oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor
Specific function:
The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
BCKDHA
Uniprot ID:
P12694
Molecular weight:
50470.58
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Gene Name:
OGDH
Uniprot ID:
Q02218
Molecular weight:
48179.59
General function:
Involved in pyridoxal phosphate binding
Specific function:
Catalyzes the last step in the trans-sulfuration pathway from methionine to cysteine. Has broad substrate specificity. Converts cystathionine to cysteine, ammonia and 2-oxobutanoate. Converts two cysteine molecules to lanthionine and hydrogen sulfide. Can also accept homocysteine as substrate. Specificity depends on the levels of the endogenous substrates. Generates the endogenous signaling molecule hydrogen sulfide (H2S), and so contributes to the regulation of blood pressure. Acts as a cysteine-protein sulfhydrase by mediating sulfhydration of target proteins: sulfhydration consists of converting -SH groups into -SSH on specific cysteine residues of target proteins such as GAPDH, PTPN1 and NF-kappa-B subunit RELA, thereby regulating their function.
Gene Name:
CTH
Uniprot ID:
P32929
Molecular weight:
41259.91
Reactions
L-Cystathionine + Water → L-Cysteine + Ammonia + 2-Ketobutyric aciddetails
Selenomethionine + Water → Methaneselenol + Ammonia + 2-Ketobutyric aciddetails
Selenocystathionine + Water → Selenocysteine + Ammonia + 2-Ketobutyric aciddetails
General function:
Involved in catalytic activity
Specific function:
Not Available
Gene Name:
SDS
Uniprot ID:
P20132
Molecular weight:
34625.105
Reactions
L-Threonine → 2-Ketobutyric acid + Ammoniadetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Displays an lactate dehydrogenase activity. Significantly increases the transcriptional activity of JUN, when overexpressed.
Gene Name:
LDHAL6A
Uniprot ID:
Q6ZMR3
Molecular weight:
36507.015
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in urocanate hydratase activity
Specific function:
Not Available
Gene Name:
UROC1
Uniprot ID:
Q96N76
Molecular weight:
80911.275
General function:
Involved in ATP binding
Specific function:
Catalyzes the phosphorylation and inactivation of the branched-chain alpha-ketoacid dehydrogenase complex, the key regulatory enzyme of the valine, leucine and isoleucine catabolic pathways. Key enzyme that regulate the activity state of the BCKD complex
Gene Name:
BCKDK
Uniprot ID:
O14874
Molecular weight:
46360.0
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHB
Uniprot ID:
P07195
Molecular weight:
36638.225
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Possible role in sperm motility.
Gene Name:
LDHC
Uniprot ID:
P07864
Molecular weight:
36310.965
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHA
Uniprot ID:
P00338
Molecular weight:
30204.975
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidoreductase activity, acting on the CH-OH group of donors, NAD or NADP as acceptor
Specific function:
Not Available
Gene Name:
LDHAL6B
Uniprot ID:
Q9BYZ2
Molecular weight:
41942.53
Reactions
2-Hydroxybutyric acid + NAD → 2-Ketobutyric acid + NADH + Hydrogen Iondetails
General function:
Involved in oxidation reduction
Specific function:
Converts trimethyllysine (TML) into hydroxytrimethyllysine (HTML).
Gene Name:
TMLHE
Uniprot ID:
Q9NVH6
Molecular weight:
44048.895
General function:
Involved in oxidoreductase activity
Specific function:
Dioxygenase that repairs alkylated DNA and RNA containing 1-methyladenine and 3-methylcytosine by oxidative demethylation. Can also repair alkylated DNA containing 1-ethenoadenine (in vitro). Has strong preference for double-stranded DNA. Has low efficiency with single-stranded substrates. Requires molecular oxygen, alpha-ketoglutarate and iron.
Gene Name:
ALKBH2
Uniprot ID:
Q6NS38
Molecular weight:
29322.22
General function:
Involved in oxidoreductase activity
Specific function:
Dioxygenase that repairs alkylated DNA containing 1- methyladenine and 3-methylcytosine by oxidative demethylation. Has a strong preference for single-stranded DNA. May also act on RNA. Requires molecular oxygen, alpha-ketoglutarate and iron
Gene Name:
ALKBH3
Uniprot ID:
Q96Q83
Molecular weight:
33374.5
General function:
Involved in catalytic activity
Specific function:
Has low serine dehydratase and threonine dehydratase activity.
Gene Name:
SDSL
Uniprot ID:
Q96GA7
Molecular weight:
34674.01
Reactions
L-Threonine → 2-Ketobutyric acid + Ammoniadetails
General function:
Involved in oxoglutarate dehydrogenase (succinyl-transferring) activity
Specific function:
Not Available
Gene Name:
OGDHL
Uniprot ID:
Q9ULD0
Molecular weight:
Not Available
General function:
Involved in G-protein coupled receptor protein signaling pathway
Specific function:
Receptor for alpha-ketoglutarate. Seems to act exclusively through a G(q)-mediated pathway
Gene Name:
OXGR1
Uniprot ID:
Q96P68
Molecular weight:
38250.6