Showing metabocard for alpha-Ketoisovaleric acid (MMDBc0000326)

Plant
Record Information
Version1.0
StatusDetected and Quantified
Creation Date2020-12-10 18:28:46 UTC
Update Date2022-08-31 03:26:56 UTC
Metabolite IDMMDBc0000326
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
MOL3D MOLSDF3D SDFPDB3D PDBSMILESInChI

MOL for #<Metabolite:0x0000560be1c5e138>


  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
Download

3D MOL for #<Metabolite:0x0000560be1c5e138>

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
Download

3D SDF for #<Metabolite:0x0000560be1c5e138>


  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>
MMDBc0000326

> <DATABASE_NAME>
MIME

> <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

$$$$
Download

3D-SDF for #<Metabolite:0x0000560be1c5e138>

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
Download

PDB for #<Metabolite:0x0000560be1c5e138>

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
Download

3D PDB for #<Metabolite:0x0000560be1c5e138>

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
Download

SMILES for #<Metabolite:0x0000560be1c5e138>

CC(C)C(=O)C(O)=O
Download

INCHI for #<Metabolite:0x0000560be1c5e138>

InChI=1S/C5H8O3/c1-3(2)4(6)5(7)8/h3H,1-2H3,(H,7,8)
Download
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
Molecular FormulaC5H8O3
Average Mass116.1152
Monoisotopic Mass116.047344122
IUPAC Name3-methyl-2-oxobutanoic acid
Traditional Nameα-ketoisovalerate
CAS Registry NumberNot Available
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