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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2022-03-07 02:49:05 UTC

HMDB ID

HMDB0000688

Secondary Accession Numbers

HMDB00688

Metabolite Identification

Common Name

Isovalerylcarnitine

Description

Isovalerylcarnitine is the phenotypic abnormality in isovaleric acidemia (OMIM 243500 ) resulting from an accumulation of isovaleric acid, which is toxic to the central nervous system. Isovaleric acidemia is an autosomal recessive inborn error of leucine metabolism caused by a deficiency of the mitochondrial enzyme isovaleryl-CoA dehydrogenase (EC 1.3.99.10) resulting in the accumulation of derivatives of isovaleryl-CoA. It was the first organic acidemia recognized in humans and can cause significant morbidity and mortality. Early diagnosis and treatment with a protein restricted diet and supplementation with carnitine and glycine are effective in promoting normal development in severely affected individuals. An alternative pathway through glycine-N-acylase (EC 2.3.1.13) allows detoxification by producing isovalerylglycine, which is excreted. Thus, isovalerylcarnitine and isovalerylglycine are the hallmarks of this disorder in plasma and urine, respectively, and are elevated regardless of a patient's metabolic condition (PMID: 16602101 ). Moreover, isovalerylcarnitine is found to be associated with celiac disease and very long-chain acyl-CoA dehydrogenase deficiency (VLCAD), which are also inborn errors of metabolism.

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HMDB0000688
     RDKit          3D
Isovalerylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    5.2601    0.3738    0.3198 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.7995    0.5926    0.6077 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.2323    1.3986   -0.5510 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1031   -0.7494    0.7117 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6332   -0.5400    0.9971 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3131    0.0115    2.0707 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6517   -0.9262    0.1279 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7549   -0.6997    0.4238 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3573   -2.0765    0.2890 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1021   -2.5534   -1.1187 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0795   -2.5514   -1.5838 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1265   -3.0002   -1.9466 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3499    0.1891   -0.6252 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7042    0.5630   -0.4807 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.6404   -0.1792   -1.3126 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2259    0.6166    0.8531 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8028    1.9734   -0.9658 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.7869    1.3023    0.0471 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4017   -0.3804   -0.4846 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7148   -0.0205    1.2644 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7063    1.1015    1.5688 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4662    0.8691   -1.4948 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1379    1.4630   -0.4937 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7207    2.3917   -0.5385 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2576   -1.3939   -0.1464 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.5010   -1.2380    1.6209 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8579   -0.2392    1.3990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8325   -2.8097    0.9655 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4415   -2.1220    0.4135 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6608    1.0794   -0.6963 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2525   -0.2825   -1.6529 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1293   -1.0199   -0.7941 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1788   -0.4942   -2.2780 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4857    0.5415   -1.5540 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3470    0.4891    0.7724 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1403    1.6642    1.2734 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8846   -0.1394    1.5499 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9872    2.5150   -0.4369 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6973    1.9607   -2.0564 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8066    2.3195   -0.6710 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  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

  Mrv1652310011918482D          

 17 16  0  0  0  0            999 V2000
   -1.7862    0.2062    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
   -1.0717    0.6188    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572    0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572    0.6188    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572    1.4438    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572    1.8563    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717    1.8563    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
   -2.1987    0.9207    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3737   -0.5082    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5006   -0.2062    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572   -0.6188    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -1.0313    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717   -0.6188    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -1.0313    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.5006   -0.6188    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -1.8563    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -1.8563    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  4  5  1  0  0  0  0
  5  6  2  0  0  0  0
  5  7  1  0  0  0  0
  1  8  1  0  0  0  0
  1  9  1  0  0  0  0
  1 10  1  0  0  0  0
  3 11  1  0  0  0  0
 11 12  1  0  0  0  0
 12 13  1  0  0  0  0
 13 14  1  0  0  0  0
 14 15  1  0  0  0  0
 14 16  1  0  0  0  0
 12 17  2  0  0  0  0
M  CHG  2   1   1   7  -1
M  END
> <DATABASE_ID>
HMDB0000688

> <DATABASE_NAME>
hmdb

> <SMILES>
CC(C)CC(=O)OC(CC([O-])=O)C[N+](C)(C)C

> <INCHI_IDENTIFIER>
InChI=1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3

> <INCHI_KEY>
IGQBPDJNUXPEMT-UHFFFAOYSA-N

> <FORMULA>
C12H23NO4

> <MOLECULAR_WEIGHT>
245.3153

> <EXACT_MASS>
245.162708229

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
40

> <JCHEM_AVERAGE_POLARIZABILITY>
26.687672380207964

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
0

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
3-[(3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate

> <ALOGPS_LOGP>
-1.95

> <JCHEM_LOGP>
-3.0142570294717457

> <ALOGPS_LOGS>
-4.08

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.342854326023389

> <JCHEM_PKA_STRONGEST_BASIC>
-7.060037223296033

> <JCHEM_POLAR_SURFACE_AREA>
66.43

> <JCHEM_REFRACTIVITY>
86.41329999999999

> <JCHEM_ROTATABLE_BOND_COUNT>
8

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.50e-02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
isovalerylcarnitine

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000688
     RDKit          3D
Isovalerylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    5.2601    0.3738    0.3198 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.7995    0.5926    0.6077 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.2323    1.3986   -0.5510 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1031   -0.7494    0.7117 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6332   -0.5400    0.9971 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3131    0.0115    2.0707 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6517   -0.9262    0.1279 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7549   -0.6997    0.4238 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3573   -2.0765    0.2890 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1021   -2.5534   -1.1187 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0795   -2.5514   -1.5838 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1265   -3.0002   -1.9466 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3499    0.1891   -0.6252 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7042    0.5630   -0.4807 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.6404   -0.1792   -1.3126 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2259    0.6166    0.8531 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8028    1.9734   -0.9658 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.7869    1.3023    0.0471 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4017   -0.3804   -0.4846 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7148   -0.0205    1.2644 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7063    1.1015    1.5688 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4662    0.8691   -1.4948 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1379    1.4630   -0.4937 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7207    2.3917   -0.5385 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2576   -1.3939   -0.1464 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.5010   -1.2380    1.6209 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8579   -0.2392    1.3990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8325   -2.8097    0.9655 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4415   -2.1220    0.4135 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6608    1.0794   -0.6963 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2525   -0.2825   -1.6529 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1293   -1.0199   -0.7941 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1788   -0.4942   -2.2780 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4857    0.5415   -1.5540 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3470    0.4891    0.7724 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1403    1.6642    1.2734 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8846   -0.1394    1.5499 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9872    2.5150   -0.4369 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6973    1.9607   -2.0564 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8066    2.3195   -0.6710 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  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

HEADER    PROTEIN                                 01-OCT-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   01-OCT-19         0                                  
HETATM    1  N   UNK     0      -3.334   0.385   0.000  0.00  0.00           N+1
HETATM    2  C   UNK     0      -2.001   1.155   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0      -0.667   0.385   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0       0.667   1.155   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       0.667   2.695   0.000  0.00  0.00           C+0
HETATM    6  O   UNK     0      -0.667   3.465   0.000  0.00  0.00           O+0
HETATM    7  O   UNK     0       2.001   3.465   0.000  0.00  0.00           O-1
HETATM    8  C   UNK     0      -4.104   1.719   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0      -2.564  -0.949   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      -4.668  -0.385   0.000  0.00  0.00           C+0
HETATM   11  O   UNK     0      -0.667  -1.155   0.000  0.00  0.00           O+0
HETATM   12  C   UNK     0       0.667  -1.925   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0       2.001  -1.155   0.000  0.00  0.00           C+0
HETATM   14  C   UNK     0       3.334  -1.925   0.000  0.00  0.00           C+0
HETATM   15  C   UNK     0       4.668  -1.155   0.000  0.00  0.00           C+0
HETATM   16  C   UNK     0       3.334  -3.465   0.000  0.00  0.00           C+0
HETATM   17  O   UNK     0       0.667  -3.465   0.000  0.00  0.00           O+0
CONECT    1    2    8    9   10                                             
CONECT    2    1    3                                                       
CONECT    3    2    4   11                                                  
CONECT    4    3    5                                                       
CONECT    5    4    6    7                                                  
CONECT    6    5                                                            
CONECT    7    5                                                            
CONECT    8    1                                                            
CONECT    9    1                                                            
CONECT   10    1                                                            
CONECT   11    3   12                                                       
CONECT   12   11   13   17                                                  
CONECT   13   12   14                                                       
CONECT   14   13   15   16                                                  
CONECT   15   14                                                            
CONECT   16   14                                                            
CONECT   17   12                                                            
MASTER        0    0    0    0    0    0    0    0   17    0   32    0
END

COMPND    HMDB0000688
HETATM    1  C1  UNL     1       5.260   0.374   0.320  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.800   0.593   0.608  1.00  0.00           C  
HETATM    3  C3  UNL     1       3.232   1.399  -0.551  1.00  0.00           C  
HETATM    4  C4  UNL     1       3.103  -0.749   0.712  1.00  0.00           C  
HETATM    5  C5  UNL     1       1.633  -0.540   0.997  1.00  0.00           C  
HETATM    6  O1  UNL     1       1.313   0.012   2.071  1.00  0.00           O  
HETATM    7  O2  UNL     1       0.652  -0.926   0.128  1.00  0.00           O  
HETATM    8  C6  UNL     1      -0.755  -0.700   0.424  1.00  0.00           C  
HETATM    9  C7  UNL     1      -1.357  -2.077   0.289  1.00  0.00           C  
HETATM   10  C8  UNL     1      -1.102  -2.553  -1.119  1.00  0.00           C  
HETATM   11  O3  UNL     1       0.080  -2.551  -1.584  1.00  0.00           O  
HETATM   12  O4  UNL     1      -2.127  -3.000  -1.947  1.00  0.00           O1-
HETATM   13  C9  UNL     1      -1.350   0.189  -0.625  1.00  0.00           C  
HETATM   14  N1  UNL     1      -2.704   0.563  -0.481  1.00  0.00           N1+
HETATM   15  C10 UNL     1      -3.640  -0.179  -1.313  1.00  0.00           C  
HETATM   16  C11 UNL     1      -3.226   0.617   0.853  1.00  0.00           C  
HETATM   17  C12 UNL     1      -2.803   1.973  -0.966  1.00  0.00           C  
HETATM   18  H1  UNL     1       5.787   1.302   0.047  1.00  0.00           H  
HETATM   19  H2  UNL     1       5.402  -0.380  -0.485  1.00  0.00           H  
HETATM   20  H3  UNL     1       5.715  -0.020   1.264  1.00  0.00           H  
HETATM   21  H4  UNL     1       3.706   1.102   1.569  1.00  0.00           H  
HETATM   22  H5  UNL     1       3.466   0.869  -1.495  1.00  0.00           H  
HETATM   23  H6  UNL     1       2.138   1.463  -0.494  1.00  0.00           H  
HETATM   24  H7  UNL     1       3.721   2.392  -0.538  1.00  0.00           H  
HETATM   25  H8  UNL     1       3.258  -1.394  -0.146  1.00  0.00           H  
HETATM   26  H9  UNL     1       3.501  -1.238   1.621  1.00  0.00           H  
HETATM   27  H10 UNL     1      -0.858  -0.239   1.399  1.00  0.00           H  
HETATM   28  H11 UNL     1      -0.832  -2.810   0.966  1.00  0.00           H  
HETATM   29  H12 UNL     1      -2.441  -2.122   0.413  1.00  0.00           H  
HETATM   30  H13 UNL     1      -0.661   1.079  -0.696  1.00  0.00           H  
HETATM   31  H14 UNL     1      -1.252  -0.282  -1.653  1.00  0.00           H  
HETATM   32  H15 UNL     1      -4.129  -1.020  -0.794  1.00  0.00           H  
HETATM   33  H16 UNL     1      -3.179  -0.494  -2.278  1.00  0.00           H  
HETATM   34  H17 UNL     1      -4.486   0.542  -1.554  1.00  0.00           H  
HETATM   35  H18 UNL     1      -4.347   0.489   0.772  1.00  0.00           H  
HETATM   36  H19 UNL     1      -3.140   1.664   1.273  1.00  0.00           H  
HETATM   37  H20 UNL     1      -2.885  -0.139   1.550  1.00  0.00           H  
HETATM   38  H21 UNL     1      -1.987   2.515  -0.437  1.00  0.00           H  
HETATM   39  H22 UNL     1      -2.697   1.961  -2.056  1.00  0.00           H  
HETATM   40  H23 UNL     1      -3.807   2.319  -0.671  1.00  0.00           H  
CONECT    1    2   18   19   20
CONECT    2    3    4   21
CONECT    3   22   23   24
CONECT    4    5   25   26
CONECT    5    6    6    7
CONECT    7    8
CONECT    8    9   13   27
CONECT    9   10   28   29
CONECT   10   11   11   12
CONECT   13   14   30   31
CONECT   14   15   16   17
CONECT   15   32   33   34
CONECT   16   35   36   37
CONECT   17   38   39   40
END

HMDB0000688
     RDKit          3D
Isovalerylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    5.2601    0.3738    0.3198 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.7995    0.5926    0.6077 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.2323    1.3986   -0.5510 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1031   -0.7494    0.7117 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6332   -0.5400    0.9971 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.3131    0.0115    2.0707 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.6517   -0.9262    0.1279 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7549   -0.6997    0.4238 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3573   -2.0765    0.2890 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1021   -2.5534   -1.1187 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0795   -2.5514   -1.5838 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1265   -3.0002   -1.9466 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3499    0.1891   -0.6252 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7042    0.5630   -0.4807 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.6404   -0.1792   -1.3126 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2259    0.6166    0.8531 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8028    1.9734   -0.9658 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.7869    1.3023    0.0471 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4017   -0.3804   -0.4846 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.7148   -0.0205    1.2644 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7063    1.1015    1.5688 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4662    0.8691   -1.4948 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1379    1.4630   -0.4937 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7207    2.3917   -0.5385 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2576   -1.3939   -0.1464 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.5010   -1.2380    1.6209 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8579   -0.2392    1.3990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8325   -2.8097    0.9655 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4415   -2.1220    0.4135 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6608    1.0794   -0.6963 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2525   -0.2825   -1.6529 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1293   -1.0199   -0.7941 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1788   -0.4942   -2.2780 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.4857    0.5415   -1.5540 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3470    0.4891    0.7724 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1403    1.6642    1.2734 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8846   -0.1394    1.5499 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9872    2.5150   -0.4369 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6973    1.9607   -2.0564 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8066    2.3195   -0.6710 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  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
3-Methylbutyrylcarnitine	ChEBI
Isovaleryl L-carnitine	HMDB
3-Methylbutyrylcarnitine, (+-)-isomer	MeSH, HMDB

Chemical Formula

C₁₂H₂₃NO₄

Average Molecular Weight

245.3153

Monoisotopic Molecular Weight

245.162708229

IUPAC Name

3-[(3-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate

Traditional Name

isovalerylcarnitine

CAS Registry Number

31023-24-2

SMILES

CC(C)CC(=O)OC(CC([O-])=O)C[N+](C)(C)C

InChI Identifier

InChI=1S/C12H23NO4/c1-9(2)6-12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3

InChI Key

IGQBPDJNUXPEMT-UHFFFAOYSA-N

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acid esters

Direct Parent

Acyl carnitines

Alternative Parents

Substituents

Acyl-carnitine
Branched fatty acid
Dicarboxylic acid or derivatives
Tetraalkylammonium salt
Quaternary ammonium salt
Carboxylic acid ester
Carboxylic acid salt
Carboxylic acid derivative
Carboxylic acid
Organonitrogen compound
Hydrocarbon derivative
Organic oxide
Organopnictogen compound
Organic nitrogen compound
Carbonyl group
Organic oxygen compound
Amine
Organooxygen compound
Organic salt
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

C5-acylcarnitine (CHEBI:73025 )

Ontology

Physiological effect

Health effect

Digestive system disorder

Celiac disease (HMDB: HMDB0000688)

Genetic disorder

Inborn errors of metabolism

Very Long Chain Acyl-CoA Dehydrogenase Deficiency (HMDB: HMDB0000688)

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 16425363)
Saliva (HMDB: HMDB0000688)

Cellular substructure

Organ

Placenta (HMDB: HMDB0000688)

Excreta

Biofluid or Excreta

Urine (PMID: 15615815)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000688)

Source

Endogenous

Endogenous (HMDB: HMDB0000688)

Animal

Animal (HMDB: HMDB0000688)

Exogenous

Food

Food (HMDB: HMDB0000688)

Animal origin

Poultry

Pheasant (FooDB: FOOD00430)
Squab (FooDB: FOOD00481)
Turkey (FooDB: FOOD00494)
Quail (FooDB: FOOD00541)
Anatidae (FooDB: FOOD00576)
Rock ptarmigan (FooDB: FOOD00597)
Columbidae (Dove, Pigeon) (FooDB: FOOD00610)
Chicken (FooDB: FOOD00329)
Mallard duck (FooDB: FOOD00353)
Emu (FooDB: FOOD00360)
Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

Deer (FooDB: FOOD00524)
Mule deer (FooDB: FOOD00348)
Elk (FooDB: FOOD00359)

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

Domestic pig (FooDB: FOOD00536)
Wild boar (FooDB: FOOD00307)

Equine

Horse (FooDB: FOOD00378)

Milk and milk product

Unfermented milk

Milk (Cow) (FooDB: FOOD00618)

Exogenous (HMDB: HMDB0000688)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0000688)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0000688)

Lipid metabolism pathway (HMDB: HMDB0000688)

Cellular process

Cell signaling (HMDB: HMDB0000688)

Biochemical process

Lipid transport (HMDB: HMDB0000688)

Role

Biological role

Energy source (HMDB: HMDB0000688)

Industrial application

Manufacturing industry

Material processing agent

Fluid processing agent

Surfactant

Surfactant (HMDB: HMDB0000688)

Pharmaceutical industry

Biomarker

Celiac disease (MarkerDB: MDB00000219)
Isovaleric acidemia (MarkerDB: MDB00000219)

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	0.025 g/L	ALOGPS
logP	-2	ALOGPS
logP	-3	ChemAxon
logS	-4.1	ALOGPS
pKa (Strongest Acidic)	4.34	ChemAxon
pKa (Strongest Basic)	-7.1	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	0	ChemAxon
Polar Surface Area	66.43 Å²	ChemAxon
Rotatable Bond Count	8	ChemAxon
Refractivity	86.41 m³·mol⁻¹	ChemAxon
Polarizability	26.69 Å³	ChemAxon
Number of Rings	0	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
AllCCS	[M+H]+	159.374	32859911
AllCCS	[M-H]-	164.532	32859911
DeepCCS	[M+H]+	159.056	30932474
DeepCCS	[M-H]-	156.698	30932474
DeepCCS	[M-2H]-	189.581	30932474
DeepCCS	[M+Na]+	165.149	30932474
AllCCS	[M+H]+	159.4	32859911
AllCCS	[M+H-H2O]+	156.4	32859911
AllCCS	[M+NH4]+	162.2	32859911
AllCCS	[M+Na]+	163.0	32859911
AllCCS	[M-H]-	164.5	32859911
AllCCS	[M+Na-2H]-	165.7	32859911
AllCCS	[M+HCOO]-	166.9	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Isovalerylcarnitine	CC(C)CC(=O)OC(CC([O-])=O)C[N+](C)(C)C	1907.5	Standard polar	33892256
Isovalerylcarnitine	CC(C)CC(=O)OC(CC([O-])=O)C[N+](C)(C)C	1296.4	Standard non polar	33892256
Isovalerylcarnitine	CC(C)CC(=O)OC(CC([O-])=O)C[N+](C)(C)C	1514.8	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylcarnitine GC-MS (Non-derivatized) - 70eV, Positive	splash10-00di-9100000000-a1c99b0f9aba5b685536	2021-09-23	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylcarnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isovalerylcarnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isovalerylcarnitine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-000j-9380000000-0e5c9a52b85b5dffea5b	2012-07-24	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylcarnitine 10V, Positive-QTOF	splash10-0002-0090000000-a919a7a98ddb53d7bb2b	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylcarnitine 20V, Positive-QTOF	splash10-000j-9050000000-87beaf2642b8fc410f0c	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isovalerylcarnitine 40V, Positive-QTOF	splash10-000i-9000000000-e9262cbaff8cb4ad0ba6	2021-09-22	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, CD₃OD, experimental)	2012-12-05	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Blood
Saliva
Urine

Tissue Locations

Placenta

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	<0.400 uM	Not Specified	Not Specified	Normal	10832746	details
Blood	Detected and Quantified	0.138 +/- 0.010 uM	Adult (>18 years old)	Both	Normal	16425363	details
Blood	Detected and Quantified	<0.600 uM	Not Specified	Not Specified	Normal	12837870	details
Blood	Detected and Quantified	0.46 uM	Newborn (0-30 days old)	Not Specified	Normal	15615815	details
Blood	Detected and Quantified	<0.110 uM	Not Specified	Not Specified	Normal	16148061	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	0.049 (0.032-0.082) uM	Children (1-13 years old)	Both	Normal	9034211	details
Saliva	Detected and Quantified	0.073 +/- 0.035 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected and Quantified	<3.0 umol/mmol creatinine	Newborn (0-30 days old)	Not Specified	Normal	15615815	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.111 +/- 0.010 uM	Adult (>18 years old)	Both	Celiac disease	16425363	details
Blood	Detected and Quantified	27.300-48.200 uM	Not Specified	Not Specified	Isovaleric acidemia	12837870	details
Blood	Detected and Quantified	0.052 (0.022-0.068) uM	Adult (>18 years old)	Both	Very long-chain acyl-CoA dehydrogenase deficiency (vLCAD)	9034211	details

Associated Disorders and Diseases

Disease References

Celiac disease
Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed:16425363 ]
Very Long Chain Acyl-CoA Dehydrogenase Deficiency
Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida I, Duran M, Jakobs C: Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res. 1997 Jan;38(1):173-82. [PubMed:9034211 ]
Isovaleric acidemia
Matern D, He M, Berry SA, Rinaldo P, Whitley CB, Madsen PP, van Calcar SC, Lussky RC, Andresen BS, Wolff JA, Vockley J: Prospective diagnosis of 2-methylbutyryl-CoA dehydrogenase deficiency in the Hmong population by newborn screening using tandem mass spectrometry. Pediatrics. 2003 Jul;112(1 Pt 1):74-8. [PubMed:12837870 ]

Associated OMIM IDs

212750 (Celiac disease)
201475 (Very Long Chain Acyl-CoA Dehydrogenase Deficiency)
243500 (Isovaleric acidemia)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022183

KNApSAcK ID

Not Available

Chemspider ID

4932271

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

Not Available

PDB ID

Not Available

ChEBI ID

73025

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000219

Good Scents ID

Not Available

References

Synthesis Reference

Nakanishi, Toyofumi; Shimizu, Akira; Arimoto, Masao; Kanai, Michiko. Synthesis of acylcarnitines for differential diagnosis of metabolic disorders. Nippon Iyo Masu Supekutoru Gakkai Koenshu (1993), 18 129-32.

Material Safety Data Sheet (MSDS)

Not Available

General References

Salamino F, Di Lisa F, Burlina AB, Menabo R, Barbato R, De Tullio R, Siliprandi N: Involvement of erythrocyte calpain in glycine- and carnitine-treated isovaleric acidemia. Pediatr Res. 1994 Aug;36(2):182-6. [PubMed:7970932 ]
Fries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S: Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. J Pediatr. 1996 Sep;129(3):449-52. [PubMed:8804338 ]
Silva MF, Selhorst J, Overmars H, van Gennip AH, Maya M, Wanders RJ, de Almeida IT, Duran M: Characterization of plasma acylcarnitines in patients under valproate monotherapy using ESI-MS/MS. Clin Biochem. 2001 Nov;34(8):635-8. [PubMed:11849623 ]
Vockley J, Ensenauer R: Isovaleric acidemia: new aspects of genetic and phenotypic heterogeneity. Am J Med Genet C Semin Med Genet. 2006 May 15;142C(2):95-103. [PubMed:16602101 ]
Brunk E, Sahoo S, Zielinski DC, Altunkaya A, Drager A, Mih N, Gatto F, Nilsson A, Preciat Gonzalez GA, Aurich MK, Prlic A, Sastry A, Danielsdottir AD, Heinken A, Noronha A, Rose PW, Burley SK, Fleming RMT, Nielsen J, Thiele I, Palsson BO: Recon3D enables a three-dimensional view of gene variation in human metabolism. Nat Biotechnol. 2018 Mar;36(3):272-281. doi: 10.1038/nbt.4072. Epub 2018 Feb 19. [PubMed:29457794 ]
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 ]

Showing metabocard for Isovalerylcarnitine (HMDB0000688)

MOL for HMDB0000688 (Isovalerylcarnitine)

3D MOL for HMDB0000688 (Isovalerylcarnitine)

3D SDF for HMDB0000688 (Isovalerylcarnitine)

3D-SDF for HMDB0000688 (Isovalerylcarnitine)

PDB for HMDB0000688 (Isovalerylcarnitine)

3D PDB for HMDB0000688 (Isovalerylcarnitine)

SMILES for HMDB0000688 (Isovalerylcarnitine)

INCHI for HMDB0000688 (Isovalerylcarnitine)

Structure for HMDB0000688 (Isovalerylcarnitine)

3D Structure for HMDB0000688 (Isovalerylcarnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra