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Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2020-11-09 23:11:34 UTC
HMDB IDHMDB0000005
Secondary Accession Numbers
  • HMDB00005
  • HMDB0006544
  • HMDB06544
Metabolite Identification
Common Name2-Ketobutyric acid
Description2-Ketobutyric acid, also known as alpha-ketobutyrate or 2-oxobutyrate, belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms. 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. It is also one of the degradation products of threonine. It can be converted into propionyl-CoA (and subsequently methylmalonyl CoA, which can be converted into succinyl CoA, a citric acid cycle intermediate), and thus enter the citric acid cycle. More specifically, 2-ketobutyric acid is a product of the lysis of cystathionine.
Structure
Data?1582752107
Synonyms
ValueSource
2-Ketobutanoic acidChEBI
2-Oxobutyric acidChEBI
3-Methyl pyruvic acidChEBI
alpha-KetobutyrateChEBI
alpha-Ketobutyric acidChEBI
alpha-oxo-N-Butyric acidChEBI
2-OxobutyrateKegg
2-Oxobutanoic acidKegg
2-KetobutanoateGenerator
3-Methyl pyruvateGenerator
a-KetobutyrateGenerator
a-Ketobutyric acidGenerator
Α-ketobutyrateGenerator
Α-ketobutyric acidGenerator
a-oxo-N-ButyrateGenerator
a-oxo-N-Butyric acidGenerator
alpha-oxo-N-ButyrateGenerator
Α-oxo-N-butyrateGenerator
Α-oxo-N-butyric acidGenerator
2-OxobutanoateGenerator
2-KetobutyrateGenerator
2-oxo-ButanoateHMDB
2-oxo-Butanoic acidHMDB
2-oxo-ButyrateHMDB
2-oxo-Butyric acidHMDB
2-oxo-N-ButyrateHMDB
2-oxo-N-Butyric acidHMDB
3-MethylpyruvateHMDB
3-Methylpyruvic acidHMDB
a-Keto-N-butyrateHMDB
a-Keto-N-butyric acidHMDB
a-OxobutyrateHMDB
a-Oxobutyric acidHMDB
alpha-Keto-N-butyrateHMDB
alpha-Keto-N-butyric acidHMDB
alpha-Ketobutric acidHMDB
alpha-OxobutyrateHMDB
alpha-Oxobutyric acidHMDB
Methyl-pyruvateHMDB
Methyl-pyruvic acidHMDB
Propionyl-formateHMDB
Propionyl-formic acidHMDB
alpha-Ketobutyric acid, sodium saltHMDB
2-Ketobutyric acidChEBI
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
Description Belongs to the class of organic compounds known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassKeto acids and derivatives
Sub ClassShort-chain keto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Short-chain keto acid
  • 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

Adverse health effect

Disposition

Biological location

Source

Route of exposure

Process

Naturally occurring process

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point33 °CNot Available
Boiling PointNot AvailableNot Available
Water Solubility119 mg/mLNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility79.2 g/LALOGPS
logP10(0.07) g/LALOGPS
logP10(0.77) g/LChemAxon
logS10(-0.11) g/LALOGPS
pKa (Strongest Acidic)3.19ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity22.62 m³·mol⁻¹ChemAxon
Polarizability9.2 ųChemAxon
Number of Rings0ChemAxon
BioavailabilityYesChemAxon
Rule of FiveYesChemAxon
Ghose FilterNoChemAxon
Veber's RuleNoChemAxon
MDDR-like RuleNoChemAxon
Predicted Chromatographic Properties

Predicted Collision Cross Sections

PredictorAdduct TypeCCS Value (Å2)Reference
DarkChem[M+H]+121.34231661259
DarkChem[M-H]-114.49731661259
AllCCS[M+H]+126.28832859911
AllCCS[M-H]-123.11832859911
DeepCCS[M+H]+126.74630932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
2-Ketobutyric acidCCC(=O)C(O)=O1676.3Standard polar33892256
2-Ketobutyric acidCCC(=O)C(O)=O1281.8Standard non polar33892256
2-Ketobutyric acidCCC(=O)C(O)=O873.7Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
2-Ketobutyric acid,1TMS,isomer #1CCC(=O)C(=O)O[Si](C)(C)C1013.7Semi standard non polar33892256
2-Ketobutyric acid,1TMS,isomer #2CC=C(O[Si](C)(C)C)C(=O)O1091.1Semi standard non polar33892256
2-Ketobutyric acid,2TMS,isomer #1CC=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1182.5Semi standard non polar33892256
2-Ketobutyric acid,2TMS,isomer #1CC=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1154.8Standard non polar33892256
2-Ketobutyric acid,2TMS,isomer #1CC=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1200.1Standard polar33892256
2-Ketobutyric acid,1TBDMS,isomer #1CCC(=O)C(=O)O[Si](C)(C)C(C)(C)C1240.5Semi standard non polar33892256
2-Ketobutyric acid,1TBDMS,isomer #2CC=C(O[Si](C)(C)C(C)(C)C)C(=O)O1332.4Semi standard non polar33892256
2-Ketobutyric acid,2TBDMS,isomer #1CC=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1617.2Semi standard non polar33892256
2-Ketobutyric acid,2TBDMS,isomer #1CC=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1574.5Standard non polar33892256
2-Ketobutyric acid,2TBDMS,isomer #1CC=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1537.5Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-0gbj-1900000000-8daa299f5bc38a7f9b3e2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-014s-1910000000-d01bcd7b41fdcc634eea2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (1 MEOX; 1 TMS)splash10-000i-9200000000-3b3816ffee57712159042014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (1 MEOX; 1 TMS)splash10-000i-6910000000-5b264d5f7e53f3e80b122014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (2 TMS)splash10-001i-6950000000-db7f6a0f03a8cce59bae2014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid EI-B (Non-derivatized)splash10-0ftb-0920000000-a6c93d8761ef15f7f7d42017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid EI-B (Non-derivatized)splash10-014i-0920000000-406fe70e72dbd9c3f2742017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Non-derivatized)splash10-0gbj-1900000000-8daa299f5bc38a7f9b3e2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Non-derivatized)splash10-014s-1910000000-d01bcd7b41fdcc634eea2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (Non-derivatized)splash10-000i-9200000000-3b3816ffee57712159042017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (Non-derivatized)splash10-000i-6910000000-5b264d5f7e53f3e80b122017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-MS (Non-derivatized)splash10-001i-6950000000-db7f6a0f03a8cce59bae2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Non-derivatized)splash10-000i-8900000000-97bab6630de4df095a7f2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - 2-Ketobutyric acid GC-EI-TOF (Non-derivatized)splash10-000i-9200000000-0ebb2354fe3f935fe1772017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-0a6r-9000000000-32269ef3ef778c482f992016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (1 TMS) - 70eV, Positivesplash10-0adi-9200000000-f92941e9f326d8ccf3e42017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - 2-Ketobutyric acid GC-MS (TBDMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum

MS/MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid Quattro_QQQ 10V, Negative-QTOF (Annotated)splash10-0pb9-9600000000-53ebb7164f74d7d92a4d2012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid Quattro_QQQ 25V, Negative-QTOF (Annotated)splash10-0zfr-5900000000-1db864754705beb79b552012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid Quattro_QQQ 40V, Negative-QTOF (Annotated)splash10-0udi-9800000000-5ba5b9ede5b1bc389b872012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid LC-ESI-QTOF (UPLC Q-Tof Premier, Waters) , Negative-QTOFsplash10-0udi-0900000000-bfdcad5bc3ed586bdede2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid LC-ESI-QTOF , negative-QTOFsplash10-0udi-0900000000-bfdcad5bc3ed586bdede2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid QqQ 4V, positive-QTOFsplash10-0pb9-9600000000-b10635fbec5572d4d0842020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid QqQ 8V, positive-QTOFsplash10-0a4i-9100000000-4c5e7d24dca6677e15702020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid QqQ 12V, positive-QTOFsplash10-0a4i-9000000000-ded44cfcbe7960954fdf2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid QqQ 16V, positive-QTOFsplash10-0a4i-9000000000-1b350be7438b08f1946a2020-07-22HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - 2-Ketobutyric acid QqQ 20V, positive-QTOFsplash10-056r-9000000000-172a175b8b9ebfd5529f2020-07-22HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 10V, Positive-QTOFsplash10-0k9i-9400000000-0061f4635858d851f1272015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 20V, Positive-QTOFsplash10-0a4r-9100000000-b943445194fb5f8e3a0f2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 40V, Positive-QTOFsplash10-0a4i-9000000000-bc5920b9bd140be6b06d2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 10V, Negative-QTOFsplash10-0udi-5900000000-f7dfb4554f0b9e1499c92015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 20V, Negative-QTOFsplash10-0a59-9200000000-e54106ad5bf45a9f072c2015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 40V, Negative-QTOFsplash10-0a4i-9000000000-7bbba57578cf720974e32015-05-27Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 10V, Positive-QTOFsplash10-0a4i-9000000000-ca1ed9c5aadcbdabc2a32021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 20V, Positive-QTOFsplash10-0a4i-9000000000-aced8e004436190f07b72021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 40V, Positive-QTOFsplash10-0a4i-9000000000-a3d7ddf227bd6aa91a542021-09-23Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 10V, Negative-QTOFsplash10-0pb9-9700000000-503296025148ec3143832021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 20V, Negative-QTOFsplash10-0a4i-9000000000-933b567955b5de5b17442021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 2-Ketobutyric acid 40V, Negative-QTOFsplash10-0006-9000000000-7d9db52021fefc87a8552021-09-25Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Saliva
  • Urine
Tissue LocationsNot Available
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
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
BloodDetected but not QuantifiedNot QuantifiedAdult (>18 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 QuantifiedAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified9.476 +/- 6.626 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Normal
    • Analysis of 30 no...
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
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothNormal details
UrineDetected and Quantified0-50 umol/mmol creatinineNewborn (0-30 days old)Both
Normal
details
Abnormal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified6.564 +/- 7.802 umol/mmol creatinineChildren (1 - 13 years old)Not Specified
Eosinophilic esophagitis
    • Analysis of 30 no...
details
Associated Disorders and Diseases
Disease References
Eosinophilic esophagitis
  1. Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Associated OMIM IDs
DrugBank IDDB04553
Phenol Explorer Compound IDNot Available
FooDB IDFDB030356
KNApSAcK IDC00019675
Chemspider ID57
KEGG Compound IDC00109
BioCyc ID2-OXOBUTANOATE
BiGG ID33889
Wikipedia LinkAlpha-Ketobutyric_acid
METLIN IDNot Available
PubChem Compound58
PDB IDNot Available
ChEBI ID30831
Food Biomarker OntologyNot Available
VMH ID2OBUT
MarkerDB IDNot Available
Good Scents IDNot Available
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 ]

Only showing the first 10 proteins. There are 21 proteins in total.

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

Only showing the first 10 proteins. There are 21 proteins in total.