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Record Information
Version4.0
Creation Date2005-11-16 15:48:42 UTC
Update Date2017-09-21 15:58:54 UTC
HMDB IDHMDB0000019
Secondary Accession Numbers
  • HMDB00019
  • HMDB04260
Metabolite Identification
Common NameAlpha-ketoisovaleric acid
DescriptionAlpha-ketoisovaleric acid is a branched chain organic acid which is a precursor to leucine and valine synthesis. It is also a degradation product from valine. The enzyme dihydroxy-acid dehydratase catalyzes the fourth step in the biosynthesis of isoleucine and valine, through the dehydration of 2, 3-dihydroxy-isovaleic acid into alpha-ketoisovaleric acid.
Structure
Thumb
Synonyms
ValueSource
2-keto-3-Methylbutyric acidChEBI
2-Ketoisovaleric acidChEBI
2-KetovalineChEBI
2-oxo-3-Methylbutanoic acidChEBI
2-oxo-3-Methylbutyric acidChEBI
2-Oxoisovaleric acidChEBI
3-Methyl-2-oxobutanoateChEBI
3-Methyl-2-oxobutyric acidChEBI
alpha-keto-Isovaleric acidChEBI
alpha-KetovalineChEBI
alpha-oxo-beta-MethylbutyricacidChEBI
alpha-Oxoisovaleric acidChEBI
Dimethylpyruvic acidChEBI
Isopropylglyoxylic acidChEBI
2-keto-3-MethylbutyrateGenerator
a-KetoisovalerateGenerator
a-Ketoisovaleric acidGenerator
alpha-KetoisovalerateGenerator
α-ketoisovalerateGenerator
α-ketoisovaleric acidGenerator
2-KetoisovalerateGenerator
2-oxo-3-MethylbutanoateGenerator
2-oxo-3-MethylbutyrateGenerator
2-OxoisovalerateGenerator
3-Methyl-2-oxobutanoic acidGenerator
3-Methyl-2-oxobutyrateGenerator
a-keto-IsovalerateGenerator
a-keto-Isovaleric acidGenerator
alpha-keto-IsovalerateGenerator
α-keto-isovalerateGenerator
α-keto-isovaleric acidGenerator
a-KetovalineGenerator
α-ketovalineGenerator
a-oxo-b-MethylbutyricacidGenerator
α-oxo-β-methylbutyricacidGenerator
a-OxoisovalerateGenerator
a-Oxoisovaleric acidGenerator
alpha-OxoisovalerateGenerator
α-oxoisovalerateGenerator
α-oxoisovaleric acidGenerator
DimethylpyruvateGenerator
IsopropylglyoxylateGenerator
2-Ketoisvaleric acidHMDB
2-oxo-3-Methyl-butyrateHMDB
3-Methyl-2-oxo-butanoateHMDB
3-Methyl-2-oxo-butanoic acidHMDB
3-Methyl-2-oxo-butyrateHMDB
3-Methyl-2-oxo-butyric acidHMDB
3-Methyl-2-oxobutinoateHMDB
3-Methyl-2-oxobutinoic acidHMDB
a-keto-b-MethylbutyrateHMDB
a-keto-b-Methylbutyric acidHMDB
a-oxo-b-MethylbutyrateHMDB
a-oxo-b-Methylbutyric acidHMDB
alpha-keto-beta-MethylbutyrateHMDB
alpha-keto-beta-Methylbutyric acidHMDB
alpha-oxo-beta-MethylbutyrateHMDB
alpha-oxo-beta-Methylbutyric acidHMDB
KetovalineHMDB
alpha-Ketoisovalerate, sodium saltMeSH
Calcium ketovalineMeSH
alpha-Ketoisopentanoic acidMeSH
alpha-Ketoisovalerate, calcium saltMeSH
Chemical FormulaC5H8O3
Average Molecular Weight116.1152
Monoisotopic Molecular Weight116.047344122
IUPAC Name3-methyl-2-oxobutanoic acid
Traditional Nameα-ketoisovalerate
CAS Registry Number759-05-7
SMILES
CC(C)C(=O)C(O)=O
InChI Identifier
InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
InChI KeyQHKABHOOEWYVLI-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of chemical entities known as short-chain keto acids and derivatives. These are keto acids with an alkyl chain the contains less than 6 carbon atoms.
KingdomChemical entities
Super ClassOrganic compounds
ClassOrganic acids and derivatives
Sub ClassKeto acids and derivatives
Direct ParentShort-chain keto acids and derivatives
Alternative Parents
Substituents
  • Branched fatty acid
  • Methyl-branched fatty acid
  • Short-chain keto acid
  • Alpha-keto acid
  • Fatty acyl
  • Alpha-hydroxy ketone
  • Ketone
  • Carboxylic acid derivative
  • Carboxylic acid
  • Monocarboxylic acid or derivatives
  • Carbonyl group
  • Hydrocarbon derivative
  • Organic oxygen compound
  • Organooxygen compound
  • Organic oxide
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External Descriptors
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
  • Food
Biofunction
  • Cell signaling
  • Fuel and energy storage
  • Fuel or energy source
  • Membrane integrity/stability
Application
  • Nutrients
  • Stabilizers
  • Surfactants and Emulsifiers
Cellular locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting Point31.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility30.2 mg/mLALOGPS
logP0.49ALOGPS
logP1.31ChemAxon
logS-0.59ALOGPS
pKa (Strongest Acidic)3.37ChemAxon
pKa (Strongest Basic)-9.7ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area54.37 Å2ChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.19 m3·mol-1ChemAxon
Polarizability11.04 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-000i-9500000000-ff936b879a69b5d118f8View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies)splash10-000i-8920000000-e37b37d64d43dcf763f0View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-e3995acc4818a98d0f48View in MoNA
GC-MSGC-MS Spectrum - GC-MS (1 MEOX; 1 TMS)splash10-0f79-9720000000-5d89487273e44ea61a68View in MoNA
Predicted GC-MSPredicted GC-MS Spectrum - GC-MSNot Available
GC-MSGC-MS Spectrum - EI-Bsplash10-000i-9700000000-209c737dcac7df2b198cView in MoNA
GC-MSGC-MS Spectrum - EI-Bsplash10-0f79-5920000000-759a2f01b4f52767ade2View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-000i-9500000000-ff936b879a69b5d118f8View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-000i-8920000000-e37b37d64d43dcf763f0View in MoNA
GC-MSGC-MS Spectrum - GC-MSsplash10-000i-9400000000-e3995acc4818a98d0f48View in MoNA
GC-MSGC-MS Spectrum - GC-MSsplash10-0f79-9720000000-5d89487273e44ea61a68View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-000i-7900000000-7e325567183fb56996d5View in MoNA
GC-MSGC-MS Spectrum - GC-EI-TOFsplash10-0ufr-0930000000-be90b06add7135b4a539View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-000i-9000000000-10ab58a33e9ca7dbace0View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-000i-9000000000-ad51ff01c94b6046ad64View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negativesplash10-014i-0900000000-9993174a7b1801b90ddbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negativesplash10-00xr-9500000000-293818b81e0879b6feb2View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negativesplash10-00di-9000000000-75058f27a2178b9cf121View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negativesplash10-0006-9000000000-4c20af39e8ee009d5278View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-014i-0900000000-9993174a7b1801b90ddbView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00xr-9500000000-1a58c6a6b4f5477dabddView in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-00di-9000000000-75058f27a2178b9cf121View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-QQ , negativesplash10-0006-9000000000-cc4465a47e663be66df5View in MoNA
LC-MS/MSLC-MS/MS Spectrum - LC-ESI-IT , negativesplash10-00di-9100000000-19511890852fce513a02View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Positivesplash10-00r2-9400000000-21f8c3fae79161c82099View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Positivesplash10-00dl-9000000000-5f25e41413738bb6b5d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Positivesplash10-0006-9000000000-801af00dea93fcfd637dView in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 10V, Negativesplash10-01b9-8900000000-5185c7dfc72c25069904View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 20V, Negativesplash10-00xs-9200000000-7e2a275a65197f96f5d7View in MoNA
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - 40V, Negativesplash10-0600-9000000000-0013e0ff06f9896a1337View in MoNA
1D NMR1H NMR SpectrumNot Available
1D NMR1H NMR SpectrumNot Available
1D NMR13C NMR SpectrumNot Available
2D NMR[1H,13C] 2D NMR SpectrumNot Available
2D NMR[1H,1H] 2D NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biofluid Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Location
  • Most Tissues
Pathways
NameSMPDB/PathwhizKEGG
2-Methyl-3-Hydroxybutryl CoA Dehydrogenase DeficiencyPw000061Pw000061 greyscalePw000061 simpleNot Available
3-Hydroxy-3-Methylglutaryl-CoA Lyase DeficiencyPw000063Pw000063 greyscalePw000063 simpleNot Available
3-Methylcrotonyl Coa Carboxylase Deficiency Type IPw000065Pw000065 greyscalePw000065 simpleNot Available
3-Methylglutaconic Aciduria Type IPw000066Pw000066 greyscalePw000066 simpleNot Available
3-Methylglutaconic Aciduria Type IIIPw000067Pw000067 greyscalePw000067 simpleNot Available
Displaying entries 1 - 5 of 17 in total
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified14.0 (0.0-28.0) uMChildren (1-13 years old)BothNormal details
BloodDetected and Quantified11.0 +/- 1.7 uMAdult (>18 years old)BothNormal
    • Geigy Scientific ...
details
BloodDetected but not Quantified Adult (>18 years old)Female
Normal
details
Cerebrospinal Fluid (CSF)Detected and Quantified6 +/- 3 uMAdult (>18 years old)BothNormal details
Cerebrospinal Fluid (CSF)Detected and Quantified8.2 +/- 6.8 uMAdult (>18 years old)BothNormal details
FecesDetected but not Quantified Adult (>18 years old)Both
Normal
details
SalivaDetected and Quantified0.679 +/- 0.369 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.752 +/- 0.287 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified1.06 +/- 0.436 uMAdult (>18 years old)Not Specified
Normal
    • Sugimoto et al. (...
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected but not Quantified Adult (>18 years old)Male
Normal
details
SalivaDetected and Quantified0.345 +/- 0.858 uMAdult (>18 years old)Male
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.434 +/- 0.382 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
SalivaDetected and Quantified0.656 +/- 0.399 uMAdult (>18 years old)Female
Normal
    • Sugimoto et al. (...
details
UrineDetected and Quantified0.78-1.2 umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.16 +/- 0.48 umol/mmol creatinineInfant (0-1 year old)BothNormal details
UrineDetected and Quantified0.5 (0.2-1.1) umol/mmol creatinineAdult (>18 years old)Both
Normal
details
UrineDetected and Quantified0.13 (0.00-0.54) umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.07 (0.01-0.45) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
UrineDetected and Quantified0.79-1.1 umol/mmol creatinineAdult (>18 years old)MaleNormal details
UrineDetected and Quantified0.07 (0.01-0.45) umol/mmol creatinineAdult (>18 years old)FemaleNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.7 +/- 5.5 uMAdult (>18 years old)BothHeart Transplant details
FecesDetected but not Quantified Adult (>18 years old)BothColorectal Cancer details
UrineDetected and Quantified1.00 (0.00-2.00) umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease details
UrineDetected and Quantified550.00 (300.00-800.00) umol/mmol creatinineChildren (1-13 years old)BothMaple syrup urine disease (MSUD) details
Associated Disorders and Diseases
Disease References
Maple syrup urine disease
  1. MetaGene [Link]
Associated OMIM IDs
DrugBank IDDB04074
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB012250
KNApSAcK IDC00007623
Chemspider ID48
KEGG Compound IDC00141
BioCyc ID2-KETO-ISOVALERATE
BiGG ID34011
Wikipedia LinkNot Available
NuGOwiki LinkHMDB0000019
METLIN ID5091
PubChem Compound49
PDB IDKIV
ChEBI ID16530
References
Synthesis ReferencePirrung, Michael C.; Ha, Hyun Joon; Holmes, Christopher P. Purification and inhibition of spinach a,b-dihydroxyacid dehydratase . Journal of Organic Chemistry (1989), 54(7), 1543-8.
Material Safety Data Sheet (MSDS)Download (PDF)
General References
  1. Schaefer K, von Herrath D, Erley CM, Asmus G: Calcium ketovaline as new therapy for uremic hyperphosphatemia. Miner Electrolyte Metab. 1990;16(6):362-4. [PubMed:2089249 ]
  2. Shoemaker JD, Elliott WH: Automated screening of urine samples for carbohydrates, organic and amino acids after treatment with urease. J Chromatogr. 1991 Jan 2;562(1-2):125-38. [PubMed:2026685 ]
  3. Livesey G, Lund P: Binding of branched-chain 2-oxo acids to bovine serum albumin. Biochem J. 1982 Apr 15;204(1):265-72. [PubMed:7115325 ]
  4. Shigematsu Y, Kikuchi K, Momoi T, Sudo M, Kikawa Y, Nosaka K, Kuriyama M, Haruki S, Sanada K, Hamano N, et al.: Organic acids and branched-chain amino acids in body fluids before and after multiple exchange transfusions in maple syrup urine disease. J Inherit Metab Dis. 1983;6(4):183-9. [PubMed:6422161 ]
  5. Chuang DT, Niu WL, Cox RP: Activities of branched-chain 2-oxo acid dehydrogenase and its components in skin fibroblasts from normal and classical-maple-syrup-urine-disease subjects. Biochem J. 1981 Oct 15;200(1):59-67. [PubMed:6895847 ]
  6. Lee SH, Kim SO, Chung BC: Gas chromatographic-mass spectrometric determination of urinary oxoacids using O-(2,3,4,5,6-pentafluorobenzyl)oxime-trimethylsilyl ester derivatization and cation-exchange chromatography. J Chromatogr B Biomed Sci Appl. 1998 Nov 20;719(1-2):1-7. [PubMed:9869358 ]
  7. Gallina DL, Dominguez JM, Hoschoian JC, Barrio JR: Maintenance of nitrogen balance in a young woman by substitution of -ketoisovaleric acid for valine. J Nutr. 1971 Sep;101(9):1165-7. [PubMed:5096137 ]
  8. Schauder P, Schroder K, Langenbeck U: Serum branched-chain amino and keto acid response to a protein-rich meal in man. Ann Nutr Metab. 1984;28(6):350-6. [PubMed:6393856 ]
  9. 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 ]

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. Jackson RH, Singer TP: Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids. J Biol Chem. 1983 Feb 10;258(3):1857-65. [PubMed:6822537 ]
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. Jackson RH, Singer TP: Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids. J Biol Chem. 1983 Feb 10;258(3):1857-65. [PubMed:6822537 ]
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. Jackson RH, Singer TP: Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids. J Biol Chem. 1983 Feb 10;258(3):1857-65. [PubMed:6822537 ]
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
References
  1. Jackson RH, Singer TP: Inactivation of the 2-ketoglutarate and pyruvate dehydrogenase complexes of beef heart by branched chain keto acids. J Biol Chem. 1983 Feb 10;258(3):1857-65. [PubMed:6822537 ]
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
Reactions
Alpha-ketoisovaleric acid + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine → [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2)details
Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxidedetails
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
Reactions
Alpha-ketoisovaleric acid + [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] lipoyllysine → [dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] S-(2-methylpropanoyl)dihydrolipoyllysine + CO(2)details
Alpha-ketoisovaleric acid + Thiamine pyrophosphate → 2-Methyl-1-hydroxypropyl-ThPP + Carbon dioxidedetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine.
Gene Name:
BCAT1
Uniprot ID:
P54687
Molecular weight:
38644.77
Reactions
L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
General function:
Involved in catalytic activity
Specific function:
Catalyzes the first reaction in the catabolism of the essential branched chain amino acids leucine, isoleucine, and valine. May also function as a transporter of branched chain alpha-keto acids.
Gene Name:
BCAT2
Uniprot ID:
O15382
Molecular weight:
33776.315
Reactions
L-Valine + Oxoglutaric acid → Alpha-ketoisovaleric acid + L-Glutamic aciddetails
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 ]

Transporters

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. 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 ]
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. 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 ]
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. 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 ]