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Showing metabocard for alpha-Ketoisovaleric acid (HMDB0000019)

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IdentificationTaxonomyOntologyPhysical propertiesSpectraBiological propertiesConcentrationsLinksReferencesenzymes (8)transporters (3) Show 11 proteinsXML
enzymes (8)transporters (3) Show 11 proteins
Record Information
Version5.0
StatusDetected and Quantified
Creation Date2005-11-16 15:48:42 UTC
Update Date2022-03-07 02:48:59 UTC
HMDB IDHMDB0000019
Secondary Accession Numbers
  • HMDB00019
  • HMDB0004260
  • HMDB04260
Metabolite Identification
Common Namealpha-Ketoisovaleric acid
Descriptionalpha-Ketoisovaleric acid is an abnormal metabolite that arises from the incomplete breakdown of branched-chain amino acids. alpha-Ketoisovaleric acid is a neurotoxin, an acidogen, and a metabotoxin. A neurotoxin causes damage to nerve cells and nerve tissues. An acidogen is an acidic compound that induces acidosis, which has multiple adverse effects on many organ systems. A metabotoxin is an endogenously produced metabolite that causes adverse health effects at chronically high levels. Chronically high levels of alpha-ketoisovaleric acid are associated with maple syrup urine disease. MSUD is a metabolic disorder caused by a deficiency of the branched-chain alpha-keto acid dehydrogenase complex (BCKDC), leading to a buildup of the branched-chain amino acids (leucine, isoleucine, and valine) and their toxic by-products (ketoacids) in the blood and urine. The symptoms of MSUD often show in infancy and lead to severe brain damage if untreated. MSUD may also present later depending on the severity of the disease. If left untreated in older individuals, during times of metabolic crisis, symptoms of the condition include uncharacteristically inappropriate, extreme, or erratic behaviour and moods, hallucinations, anorexia, weight loss, anemia, diarrhea, vomiting, dehydration, lethargy, oscillating hypertonia and hypotonia, ataxia, seizures, hypoglycemia, ketoacidosis, opisthotonus, pancreatitis, rapid neurological decline, and coma. In maple syrup urine disease, the brain concentration of branched-chain ketoacids can increase 10- to 20-fold. This leads to a depletion of glutamate and a consequent reduction in the concentration of brain glutamine, aspartate, alanine, and other amino acids. The result is a compromise of energy metabolism because of a failure of the malate-aspartate shuttle and a diminished rate of protein synthesis (PMID: 15930465 ). alpha-Ketoisovaleric acid is a keto-acid, which is a subclass of organic acids. Abnormally high levels of organic acids in the blood (organic acidemia), urine (organic aciduria), the brain, and other tissues lead to general metabolic acidosis. Acidosis typically occurs when arterial pH falls below 7.35. In infants with acidosis, the initial symptoms include poor feeding, vomiting, loss of appetite, weak muscle tone (hypotonia), and lack of energy (lethargy). These can progress to heart, liver, and kidney abnormalities, seizures, coma, and possibly death. These are also the characteristic symptoms of untreated MSUD. Many affected children with organic acidemias experience intellectual disability or delayed development.
Structure
Data?1582752108
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for HMDB0000019 (alpha-Ketoisovaleric acid)


  Mrv1652309032023502D          

  8  7  0  0  0  0            999 V2000
10007.706010007.5050    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10008.421410007.9171    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10009.134810007.5050    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10008.421410008.7418    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10006.991310007.9171    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10006.276510007.5050    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10006.991310008.7418    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10007.706010006.6797    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  5  1  0  0  0  0
  1  8  2  0  0  0  0
  2  3  1  0  0  0  0
  2  4  1  0  0  0  0
  5  6  1  0  0  0  0
  5  7  2  0  0  0  0
M  END
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3D MOL for HMDB0000019 (alpha-Ketoisovaleric acid)

HMDB0000019
     RDKit          3D
alpha-Ketoisovaleric acid
 16 15  0  0  0  0  0  0  0  0999 V2000
   -1.2918   -0.9728    0.2156 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5333    0.3218    0.2781 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2104    1.4542   -0.4369 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7996    0.1215   -0.3234 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.1082    0.8160   -1.3242 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.7692   -0.8274    0.1727 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4728   -1.5250    1.1693 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0127   -1.0097   -0.3941 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7572   -1.7096   -0.4402 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3063   -0.8795   -0.1752 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3188   -1.4436    1.2189 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4615    0.6365    1.3597 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4653    1.2087   -1.4903 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0918    1.8519    0.1029 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4724    2.2899   -0.4856 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7464   -0.3329   -0.4239 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  2  0
  4  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  3 13  1  0
  3 14  1  0
  3 15  1  0
  8 16  1  0
M  END
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3D SDF for HMDB0000019 (alpha-Ketoisovaleric acid)


  Mrv1652309032023502D          

  8  7  0  0  0  0            999 V2000
10007.706010007.5050    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10008.421410007.9171    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10009.134810007.5050    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10008.421410008.7418    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10006.991310007.9171    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10006.276510007.5050    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10006.991310008.7418    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10007.706010006.6797    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  5  1  0  0  0  0
  1  8  2  0  0  0  0
  2  3  1  0  0  0  0
  2  4  1  0  0  0  0
  5  6  1  0  0  0  0
  5  7  2  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000019

> <DATABASE_NAME>
hmdb

> <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_KEY>
QHKABHOOEWYVLI-UHFFFAOYSA-N

> <FORMULA>
C5H8O3

> <MOLECULAR_WEIGHT>
116.1152

> <EXACT_MASS>
116.047344122

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
16

> <JCHEM_AVERAGE_POLARIZABILITY>
11.043255629982493

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
3-methyl-2-oxobutanoic acid

> <ALOGPS_LOGP>
0.49

> <JCHEM_LOGP>
1.3093960786666665

> <ALOGPS_LOGS>
-0.59

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.3715855069017753

> <JCHEM_PKA_STRONGEST_BASIC>
-9.71868553071473

> <JCHEM_POLAR_SURFACE_AREA>
54.37

> <JCHEM_REFRACTIVITY>
27.191100000000002

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
3.02e+01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
α-ketoisovalerate

> <JCHEM_VEBER_RULE>
0

$$$$
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3D-SDF for HMDB0000019 (alpha-Ketoisovaleric acid)

HMDB0000019
     RDKit          3D
alpha-Ketoisovaleric acid
 16 15  0  0  0  0  0  0  0  0999 V2000
   -1.2918   -0.9728    0.2156 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5333    0.3218    0.2781 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2104    1.4542   -0.4369 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7996    0.1215   -0.3234 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.1082    0.8160   -1.3242 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.7692   -0.8274    0.1727 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4728   -1.5250    1.1693 O   0  0  0  0  0  0  0  0  0  0  0  0
    3.0127   -1.0097   -0.3941 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7572   -1.7096   -0.4402 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3063   -0.8795   -0.1752 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3188   -1.4436    1.2189 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4615    0.6365    1.3597 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4653    1.2087   -1.4903 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0918    1.8519    0.1029 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4724    2.2899   -0.4856 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7464   -0.3329   -0.4239 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  2  0
  4  6  1  0
  6  7  2  0
  6  8  1  0
  1  9  1  0
  1 10  1  0
  1 11  1  0
  2 12  1  0
  3 13  1  0
  3 14  1  0
  3 15  1  0
  8 16  1  0
M  END
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PDB for HMDB0000019 (alpha-Ketoisovaleric acid)

HEADER    PROTEIN                                 03-SEP-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   03-SEP-20         0                                  
HETATM    1  C   UNK     0    8681.0518680.676   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0    8682.3878681.445   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8683.7188680.676   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8682.3878682.985   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0    8679.7178681.445   0.000  0.00  0.00           C+0
HETATM    6  O   UNK     0    8678.3838680.676   0.000  0.00  0.00           O+0
HETATM    7  O   UNK     0    8679.7178682.985   0.000  0.00  0.00           O+0
HETATM    8  O   UNK     0    8681.0518679.135   0.000  0.00  0.00           O+0
CONECT    1    2    5    8                                                  
CONECT    2    1    3    4                                                  
CONECT    3    2                                                            
CONECT    4    2                                                            
CONECT    5    1    6    7                                                  
CONECT    6    5                                                            
CONECT    7    5                                                            
CONECT    8    1                                                            
MASTER        0    0    0    0    0    0    0    0    8    0   14    0
END
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3D PDB for HMDB0000019 (alpha-Ketoisovaleric acid)

COMPND    HMDB0000019
HETATM    1  C1  UNL     1      -1.292  -0.973   0.216  1.00  0.00           C  
HETATM    2  C2  UNL     1      -0.533   0.322   0.278  1.00  0.00           C  
HETATM    3  C3  UNL     1      -1.210   1.454  -0.437  1.00  0.00           C  
HETATM    4  C4  UNL     1       0.800   0.121  -0.323  1.00  0.00           C  
HETATM    5  O1  UNL     1       1.108   0.816  -1.324  1.00  0.00           O  
HETATM    6  C5  UNL     1       1.769  -0.827   0.173  1.00  0.00           C  
HETATM    7  O2  UNL     1       1.473  -1.525   1.169  1.00  0.00           O  
HETATM    8  O3  UNL     1       3.013  -1.010  -0.394  1.00  0.00           O  
HETATM    9  H1  UNL     1      -0.757  -1.710  -0.440  1.00  0.00           H  
HETATM   10  H2  UNL     1      -2.306  -0.880  -0.175  1.00  0.00           H  
HETATM   11  H3  UNL     1      -1.319  -1.444   1.219  1.00  0.00           H  
HETATM   12  H4  UNL     1      -0.462   0.637   1.360  1.00  0.00           H  
HETATM   13  H5  UNL     1      -1.465   1.209  -1.490  1.00  0.00           H  
HETATM   14  H6  UNL     1      -2.092   1.852   0.103  1.00  0.00           H  
HETATM   15  H7  UNL     1      -0.472   2.290  -0.486  1.00  0.00           H  
HETATM   16  H8  UNL     1       3.746  -0.333  -0.424  1.00  0.00           H  
CONECT    1    2    9   10   11
CONECT    2    3    4   12
CONECT    3   13   14   15
CONECT    4    5    5    6
CONECT    6    7    7    8
CONECT    8   16
END
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SMILES for HMDB0000019 (alpha-Ketoisovaleric acid)

CC(C)C(=O)C(O)=O
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INCHI for HMDB0000019 (alpha-Ketoisovaleric acid)

InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
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View in JSmolView NMR AssignmentsView Stereo Labels
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-oxo-3-MethylbutanoateKegg
2-OxoisovalerateKegg
2-OxoisopentanoateKegg
2-Keto-3-methylbutyrateGenerator
2-KetoisovalerateGenerator
2-oxo-3-MethylbutyrateGenerator
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-Oxoisopentanoic acidGenerator
a-KetoisovalerateGenerator
a-Ketoisovaleric acidGenerator
alpha-KetoisovalerateGenerator
Α-ketoisovalerateGenerator
Α-ketoisovaleric acidGenerator
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-Ketoisopentanoic acidHMDB
alpha-Ketoisovaleric acidGenerator, MeSH
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
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
  • 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
Physiological effect

Adverse health effect

Disposition

Biological location

Physical Properties
StateSolid
Experimental Molecular Properties
PropertyValueReference
Melting Point31.5 °CNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Experimental Chromatographic PropertiesNot Available
Predicted Molecular Properties
PropertyValueSource
Water Solubility30.2 g/LALOGPS
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 ŲChemAxon
Rotatable Bond Count2ChemAxon
Refractivity27.19 m³·mol⁻¹ChemAxon
Polarizability11.04 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]+124.01131661259
DarkChem[M-H]-119.55431661259
AllCCS[M+H]+127.86932859911
AllCCS[M-H]-124.4232859911
DeepCCS[M+H]+125.87830932474
DeepCCS[M-H]-123.08430932474
DeepCCS[M-2H]-159.59630932474
DeepCCS[M+Na]+134.36330932474

Predicted Kovats Retention Indices

Underivatized

MetaboliteSMILESKovats RI ValueColumn TypeReference
alpha-Ketoisovaleric acidCC(C)C(=O)C(O)=O1644.5Standard polar33892256
alpha-Ketoisovaleric acidCC(C)C(=O)C(O)=O1270.4Standard non polar33892256
alpha-Ketoisovaleric acidCC(C)C(=O)C(O)=O895.0Semi standard non polar33892256

Derivatized

Derivative Name / StructureSMILESKovats RI ValueColumn TypeReference
alpha-Ketoisovaleric acid,1TMS,isomer #1CC(C)C(=O)C(=O)O[Si](C)(C)C1039.8Semi standard non polar33892256
alpha-Ketoisovaleric acid,1TMS,isomer #2CC(C)=C(O[Si](C)(C)C)C(=O)O1112.5Semi standard non polar33892256
alpha-Ketoisovaleric acid,2TMS,isomer #1CC(C)=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1228.7Semi standard non polar33892256
alpha-Ketoisovaleric acid,2TMS,isomer #1CC(C)=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1230.3Standard non polar33892256
alpha-Ketoisovaleric acid,2TMS,isomer #1CC(C)=C(O[Si](C)(C)C)C(=O)O[Si](C)(C)C1223.5Standard polar33892256
alpha-Ketoisovaleric acid,1TBDMS,isomer #1CC(C)C(=O)C(=O)O[Si](C)(C)C(C)(C)C1270.2Semi standard non polar33892256
alpha-Ketoisovaleric acid,1TBDMS,isomer #2CC(C)=C(O[Si](C)(C)C(C)(C)C)C(=O)O1366.8Semi standard non polar33892256
alpha-Ketoisovaleric acid,2TBDMS,isomer #1CC(C)=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1654.9Semi standard non polar33892256
alpha-Ketoisovaleric acid,2TBDMS,isomer #1CC(C)=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1664.8Standard non polar33892256
alpha-Ketoisovaleric acid,2TBDMS,isomer #1CC(C)=C(O[Si](C)(C)C(C)(C)C)C(=O)O[Si](C)(C)C(C)(C)C1565.1Standard polar33892256
Spectra

GC-MS Spectra

Spectrum TypeDescriptionSplash KeyDeposition DateSourceView
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-9500000000-ff936b879a69b5d118f82014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Pegasus III TOF-MS system, Leco; GC 6890, Agilent Technologies) (Non-derivatized)splash10-000i-8920000000-e37b37d64d43dcf763f02014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (1 MEOX; 1 TMS)splash10-000i-9400000000-e3995acc4818a98d0f482014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (1 MEOX; 1 TMS)splash10-0f79-9720000000-5d89487273e44ea61a682014-06-16HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid EI-B (Non-derivatized)splash10-000i-9700000000-209c737dcac7df2b198c2017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid EI-B (Non-derivatized)splash10-0f79-5920000000-759a2f01b4f52767ade22017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Non-derivatized)splash10-000i-9500000000-ff936b879a69b5d118f82017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Non-derivatized)splash10-000i-8920000000-e37b37d64d43dcf763f02017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (Non-derivatized)splash10-000i-9400000000-e3995acc4818a98d0f482017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (Non-derivatized)splash10-0f79-9720000000-5d89487273e44ea61a682017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Non-derivatized)splash10-000i-7900000000-7e325567183fb56996d52017-09-12HMDB team, MONA, MassBankView Spectrum
Experimental GC-MSGC-MS Spectrum - alpha-Ketoisovaleric acid GC-EI-TOF (Non-derivatized)splash10-0ufr-0930000000-be90b06add7135b4a5392017-09-12HMDB team, MONA, MassBankView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (Non-derivatized) - 70eV, Positivesplash10-00dl-9000000000-52f9d8fc8386e4c512fe2016-09-22Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (1 TMS) - 70eV, Positivesplash10-006x-9600000000-52aefdc997dac92c84592017-10-06Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (Non-derivatized) - 70eV, PositiveNot Available2021-10-12Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (TMS_1_2) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric acid GC-MS (TBDMS_1_1) - 70eV, PositiveNot Available2021-11-05Wishart LabView Spectrum
Predicted GC-MSPredicted GC-MS Spectrum - alpha-Ketoisovaleric 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 - alpha-Ketoisovaleric acid Quattro_QQQ 10V, Positive-QTOF (Annotated)splash10-000i-9000000000-10ab58a33e9ca7dbace02012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid Quattro_QQQ 25V, Positive-QTOF (Annotated)splash10-000i-9000000000-ad51ff01c94b6046ad642012-07-24HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ (API3000, Applied Biosystems) 10V, Negative-QTOFsplash10-014i-0900000000-9993174a7b1801b90ddb2012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ (API3000, Applied Biosystems) 20V, Negative-QTOFsplash10-00xr-9500000000-293818b81e0879b6feb22012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ (API3000, Applied Biosystems) 30V, Negative-QTOFsplash10-00di-9000000000-75058f27a2178b9cf1212012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ (API3000, Applied Biosystems) 40V, Negative-QTOFsplash10-0006-9000000000-4c20af39e8ee009d52782012-08-31HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ , negative-QTOFsplash10-014i-0900000000-9993174a7b1801b90ddb2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ , negative-QTOFsplash10-00xr-9500000000-1a58c6a6b4f5477dabdd2017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ , negative-QTOFsplash10-00di-9000000000-75058f27a2178b9cf1212017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-QQ , negative-QTOFsplash10-0006-9000000000-cc4465a47e663be66df52017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid LC-ESI-IT , negative-QTOFsplash10-00di-9100000000-19511890852fce513a022017-09-14HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid 20V, Positive-QTOFsplash10-00kf-9300000000-30d3bdfb6d38dbb8e1012021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid 40V, Positive-QTOFsplash10-000l-9000000000-879b6502ff380673b7762021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid 10V, Positive-QTOFsplash10-0159-9800000000-0cf4d3a0e7b3356995c62021-09-20HMDB team, MONAView Spectrum
Experimental LC-MS/MSLC-MS/MS Spectrum - alpha-Ketoisovaleric acid 35V, Positive-QTOFsplash10-014o-9900000000-f35430a2eaea0d81df322021-09-20HMDB team, MONAView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 10V, Positive-QTOFsplash10-00r2-9400000000-21f8c3fae79161c820992015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 20V, Positive-QTOFsplash10-00dl-9000000000-5f25e41413738bb6b5d72015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 40V, Positive-QTOFsplash10-0006-9000000000-801af00dea93fcfd637d2015-04-24Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 10V, Negative-QTOFsplash10-01b9-8900000000-5185c7dfc72c250699042015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 20V, Negative-QTOFsplash10-00xs-9200000000-7e2a275a65197f96f5d72015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 40V, Negative-QTOFsplash10-0600-9000000000-0013e0ff06f9896a13372015-04-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 10V, Negative-QTOFsplash10-01b9-6900000000-7f00871b074cbd9a42e62021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 20V, Negative-QTOFsplash10-06di-9400000000-6d56778068e11e4f8e852021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 40V, Negative-QTOFsplash10-0a4i-9000000000-b445e7fc67a8ff2bd3ba2021-09-25Wishart LabView Spectrum
Predicted LC-MS/MSPredicted LC-MS/MS Spectrum - alpha-Ketoisovaleric acid 10V, Positive-QTOFsplash10-006x-9000000000-733fb7227d2d053b112f2021-09-25Wishart LabView Spectrum

NMR Spectra

Spectrum TypeDescriptionDeposition DateSourceView
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 100 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 200 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 300 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 400 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 500 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 600 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 700 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 800 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 900 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR1H NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Predicted 1D NMR13C NMR Spectrum (1D, 1000 MHz, D2O, predicted)2021-09-16Wishart LabView Spectrum
Experimental 1D NMR1H NMR Spectrum (1D, 500 MHz, H2O, experimental)2021-10-10Wishart LabView Spectrum
Experimental 2D NMR[1H, 13C]-HSQC NMR Spectrum (2D, 600 MHz, H2O, experimental)2012-12-04Wishart LabView Spectrum
Biological Properties
Cellular Locations
  • Cytoplasm
  • Extracellular
  • Membrane
  • Mitochondria
Biospecimen Locations
  • Blood
  • Cerebrospinal Fluid (CSF)
  • Feces
  • Saliva
  • Urine
Tissue Locations
  • Placenta
Pathways
Normal Concentrations
BiospecimenStatusValueAgeSexConditionReferenceDetails
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 QuantifiedNot QuantifiedAdult (>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 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 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 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 but not QuantifiedNot QuantifiedAdult (>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
BiospecimenStatusValueAgeSexConditionReferenceDetails
BloodDetected and Quantified10.7 +/- 5.5 uMAdult (>18 years old)BothHeart Transplant details
BloodDetected and Quantified237 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
 details
Cerebrospinal Fluid (CSF)Detected and Quantified189 uMChildren (1-13 years old)Female
Maple syrup urine disease
    • Peritoneal dialys...
 details
FecesDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothColorectal Cancer details
UrineDetected but not QuantifiedNot QuantifiedAdult (>18 years old)BothBladder cancer details
UrineDetected and Quantified550.00 (300.00-800.00) umol/mmol creatinineAdult (>18 years old)BothMaple syrup urine disease (MSUD). When treated, the concentrations are 1-2 uM/mM.
    • MetaGene: Metabol...
 details
UrineDetected and Quantified550.00 (300.00-800.00) umol/mmol creatinineChildren (1-13 years old)BothMaple syrup urine disease (MSUD). When treated, the concentrations are 1-2 uM/mM.
    • MetaGene: Metabol...
 details
Associated Disorders and Diseases
Disease References
Colorectal cancer
  1. Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]
Maple syrup urine disease
  1. G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
  2. Wendel, U., Becker, K., Przyrembel, H. et al. (1980). Peritoneal dialysis in maple-syrup-urine disease: Studies on branched-chain amino and keto acids. Eur J Pediatr (1980) 134: 57. https://doi.org/10.1007/BF00442404. Eur J Pediatr.
Associated OMIM IDs
DrugBank IDDB04074
Phenol Explorer Compound IDNot Available
FooDB IDFDB012250
KNApSAcK IDC00007623
Chemspider ID48
KEGG Compound IDC00141
BioCyc ID2-KETO-ISOVALERATE
BiGG ID34011
Wikipedia LinkAlpha-Ketoisovaleric acid
METLIN ID5091
PubChem Compound49
PDB IDNot Available
ChEBI ID16530
Food Biomarker OntologyNot Available
VMH ID3MOB
MarkerDB IDMDB00000009
Good Scents IDNot Available
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 ]
  10. Yudkoff M, Daikhin Y, Nissim I, Horyn O, Luhovyy B, Lazarow A, Nissim I: Brain amino acid requirements and toxicity: the example of leucine. J Nutr. 2005 Jun;135(6 Suppl):1531S-8S. [PubMed:15930465 ]
  11. 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 11 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
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 ]
Enzyme Details
2. 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
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 ]
Enzyme Details
3. 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
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 ]
Enzyme Details
4. 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
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 ]
Enzyme Details
5. 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
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
Enzyme Details
6. 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
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
Enzyme Details
7. Branched-chain-amino-acid aminotransferase, cytosolic
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
Enzyme Details
8. Branched-chain-amino-acid aminotransferase, mitochondrial
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

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. 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. 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 ]
Enzyme Details
3. 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. 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 ]

Only showing the first 10 proteins. There are 11 proteins in total.

Show all enzymes and transporters