NP-MRD: Showing NP-Card for Isovalerylcarnitine (NP0001190)

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Record Information

Version

2.0

Created at

2005-11-16 15:48:42 UTC

Updated at

2024-09-17 15:44:27 UTC

NP-MRD ID

NP0001190

Secondary Accession Numbers

None

Natural Product 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>
NP0001190

> <DATABASE_NAME>
NP-MRD

> <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

Synonyms

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

Chemical Formula

C₁₂H₂₃NO₄

Average Mass

245.3153 Da

Monoisotopic Mass

245.16271 Da

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

Experimental Spectra

Spectrum Type	Description	Depositor Email	Depositor Organization	Depositor	Deposition Date	View
2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, CD₃OD, experimental)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum

Predicted Spectra

Spectrum Type	Description	Depositor ID	Depositor Organization	Depositor	Deposition Date	View
1D NMR	¹³C NMR Spectrum (1D, 25 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 252 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 50 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 75 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 101 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 126 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 151 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 176 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 201 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 226 MHz, D₂O, predicted)	Wishart Lab	Wishart Lab	David Wishart	2021-06-20	View Spectrum

Chemical Shift Submissions

Not Available

Species

Species of Origin

Species Name	Source	Reference
Anas platyrhynchos	FooDB	FooDB
Anatidae	FooDB	FooDB
Anser anser	FooDB	FooDB
Bison bison	FooDB	FooDB
Bos taurus	FooDB	FooDB
Bos taurus X Bison bison	FooDB	FooDB
Bubalus bubalis	FooDB	FooDB
Capra aegagrus hircus	FooDB	FooDB
Cervidae	FooDB	FooDB
Cervus canadensis	FooDB	FooDB
Columba	FooDB	FooDB
Columbidae	FooDB	FooDB
Dromaius novaehollandiae	FooDB	FooDB
Equus caballus	FooDB	FooDB
Gallus gallus	FooDB	FooDB
Lagopus muta	FooDB	FooDB
Leporidae	FooDB	FooDB
Lepus timidus	FooDB	FooDB
Melanitta fusca	FooDB	FooDB
Meleagris gallopavo	FooDB	FooDB
Numida meleagris	FooDB	FooDB
Odocoileus	FooDB	FooDB
Oryctolagus	FooDB	FooDB
Ovis aries	FooDB	FooDB
Phasianidae	FooDB	FooDB
Phasianus colchicus	FooDB	FooDB
Struthio camelus	FooDB	FooDB
Sus scrofa	FooDB	FooDB
Sus scrofa domestica	FooDB	FooDB

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 )

Physical Properties

State

Solid

Experimental 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

Predicted 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

External Links

HMDB ID

HMDB0000688

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FoodDB 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

5657

PubChem Compound

6426851

PDB ID

Not Available

ChEBI ID

73025

Good Scents ID

Not Available

References

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 ]

Showing NP-Card for Isovalerylcarnitine (NP0001190)

MOL for NP0001190 (Isovalerylcarnitine)

3D MOL for NP0001190 (Isovalerylcarnitine)

3D SDF for NP0001190 (Isovalerylcarnitine)

3D-SDF for NP0001190 (Isovalerylcarnitine)

PDB for NP0001190 (Isovalerylcarnitine)

3D PDB for NP0001190 (Isovalerylcarnitine)

SMILES for NP0001190 (Isovalerylcarnitine)

INCHI for NP0001190 (Isovalerylcarnitine)

Structure for NP0001190 (Isovalerylcarnitine)

3D Structure for NP0001190 (Isovalerylcarnitine)