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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2009-11-03 00:25:37 UTC

Update Date

2023-05-30 20:55:50 UTC

HMDB ID

HMDB0013132

Secondary Accession Numbers

HMDB13132

Metabolite Identification

Common Name

Hydroxyvalerylcarnitine

Description

Hydroxyvalerylcarnitine is an acylcarnitine. More specifically, it is an hydroxyvaleric acid ester of carnitine. Acylcarnitines were first discovered more than 70 year ago (PMID: 13825279 ). It is believed that there are more than 1000 types of acylcarnitines in the human body. The general role of acylcarnitines is to transport acyl-groups (organic acids and fatty acids) from the cytoplasm into the mitochondria so that they can be broken down to produce energy. This process is known as beta-oxidation. According to a recent review (PMID: 35710135 ), acylcarnitines (ACs) can be classified into 9 different categories depending on the type and size of their acyl-group: 1) short-chain ACs; 2) medium-chain ACs; 3) long-chain ACs; 4) very long-chain ACs; 5) hydroxy ACs; 6) branched chain ACs; 7) unsaturated ACs; 8) dicarboxylic ACs and 9) miscellaneous ACs. Short-chain ACs have acyl-groups with two to five carbons (C2-C5), medium-chain ACs have acyl-groups with six to thirteen carbons (C6-C13), long-chain ACs have acyl-groups with fourteen to twenty once carbons (C14-C21) and very long-chain ACs have acyl groups with more than 22 carbons. Hydroxyvalerylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine hydroxyvalerylcarnitine is a member of the most abundant group of carnitines in the body, comprising more than 50% of all acylcarnitines quantified in tissues and biofluids (PMID: 31920980 ). Some short-chain carnitines have been studied as supplements or treatments for a number of diseases, including neurological disorders and inborn errors of metabolism. In particular hydroxyvalerylcarnitine is elevated in the blood or plasma of individuals with 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency (PMID: 28583327 ) and holocarboxylase synthetase deficiency (PMID: 27114915 ). It is also decreased in the blood or plasma of individuals with psoriasis (PMID: 33391503 ). Carnitine acetyltransferase (CrAT, EC:2.3.1.7) is responsible for the synthesis of all short-chain and short branched-chain acylcarnitines (PMID: 23485643 ). The study of acylcarnitines is an active area of research and it is likely that many novel acylcarnitines will be discovered in the coming years. It is also likely that many novel roles in health and disease will be uncovered. An excellent review of the current state of knowledge for acylcarnitines is available (PMID: 35710135 ).

Structure

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

HMDB0013132
     RDKit          3D
Hydroxyvalerylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    4.0271   -1.6948   -0.2977 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5558   -1.3185    1.0966 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8518    0.0142    0.9778 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3948    0.4129    2.2305 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.6449   -0.1992    0.0789 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9444    1.1243   -0.0404 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.9456    1.9521   -0.6059 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1460    1.0024   -0.7701 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3761    1.3430   -0.4811 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.9738    2.5147   -1.2545 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1851    3.7540   -1.0402 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1310    3.9699   -1.6901 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5621    4.7268   -0.1250 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3200    0.2026   -0.6429 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1749   -0.9590    0.1254 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.4207   -1.9604   -0.6528 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6160   -0.8943    1.4206 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5050   -1.6124    0.2829 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2345   -0.7939   -0.9155 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9577   -2.2900   -0.2730 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2428   -2.2711   -0.8228 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.3679   -1.2984    1.8259 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7599   -2.0587    1.3975 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4962    0.7972    0.5696 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1087    0.7300    2.8227 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0352   -1.0379    0.3931 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.0797   -0.4293   -0.9401 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.7722    1.5251    0.9635 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.8466    1.8962   -1.5894 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4399    1.7266    0.5732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1629    2.2974   -2.2974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9642    2.7133   -0.7905 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3750   -0.1004   -1.7469 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3968    0.5656   -0.4974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0860   -2.4869   -1.3427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8574   -2.6269    0.0055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6757   -1.3668   -1.2590 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4617   -0.9192    2.1732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1040    0.0651    1.6713 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9920   -1.7722    1.7010 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9054   -1.6906   -0.7458 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1340   -0.9597    0.9250 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3003   -2.5930    0.7278 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  6  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  1  0
 11 12  2  0
 11 13  1  0
  9 14  1  0
 14 15  1  0
 15 16  1  0
 15 17  1  0
 15 18  1  0
  1 19  1  0
  1 20  1  0
  1 21  1  0
  2 22  1  0
  2 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  5 27  1  0
  6 28  1  0
  7 29  1  0
  9 30  1  1
 10 31  1  0
 10 32  1  0
 14 33  1  0
 14 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
 18 41  1  0
 18 42  1  0
 18 43  1  0
M  CHG  2  13  -1  15   1
M  END

HMDB0013132
     RDKit          3D
Hydroxyvalerylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    4.0271   -1.6948   -0.2977 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5558   -1.3185    1.0966 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8518    0.0142    0.9778 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3948    0.4129    2.2305 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.6449   -0.1992    0.0789 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9444    1.1243   -0.0404 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.9456    1.9521   -0.6059 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1460    1.0024   -0.7701 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3761    1.3430   -0.4811 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.9738    2.5147   -1.2545 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1851    3.7540   -1.0402 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1310    3.9699   -1.6901 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5621    4.7268   -0.1250 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3200    0.2026   -0.6429 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1749   -0.9590    0.1254 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.4207   -1.9604   -0.6528 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6160   -0.8943    1.4206 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5050   -1.6124    0.2829 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2345   -0.7939   -0.9155 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9577   -2.2900   -0.2730 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2428   -2.2711   -0.8228 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.3679   -1.2984    1.8259 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7599   -2.0587    1.3975 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4962    0.7972    0.5696 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1087    0.7300    2.8227 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0352   -1.0379    0.3931 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.0797   -0.4293   -0.9401 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.7722    1.5251    0.9635 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.8466    1.8962   -1.5894 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4399    1.7266    0.5732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1629    2.2974   -2.2974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9642    2.7133   -0.7905 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3750   -0.1004   -1.7469 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3968    0.5656   -0.4974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0860   -2.4869   -1.3427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8574   -2.6269    0.0055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6757   -1.3668   -1.2590 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4617   -0.9192    2.1732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1040    0.0651    1.6713 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9920   -1.7722    1.7010 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9054   -1.6906   -0.7458 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1340   -0.9597    0.9250 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3003   -2.5930    0.7278 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  6  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  1  0
 11 12  2  0
 11 13  1  0
  9 14  1  0
 14 15  1  0
 15 16  1  0
 15 17  1  0
 15 18  1  0
  1 19  1  0
  1 20  1  0
  1 21  1  0
  2 22  1  0
  2 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  5 27  1  0
  6 28  1  0
  7 29  1  0
  9 30  1  1
 10 31  1  0
 10 32  1  0
 14 33  1  0
 14 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
 18 41  1  0
 18 42  1  0
 18 43  1  0
M  CHG  2  13  -1  15   1
M  END

HEADER    PROTEIN                                 25-FEB-21   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   25-FEB-21         0                                  
HETATM    1  C   UNK     0      21.535  -8.947   0.000  0.00  0.00           C+0
HETATM    2  C   UNK     0      22.869  -8.177   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0      24.202  -8.947   0.000  0.00  0.00           C+0
HETATM    4  O   UNK     0      24.202 -10.487   0.000  0.00  0.00           O-1
HETATM    5  O   UNK     0      21.535 -10.487   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0      25.536  -8.177   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0      20.202  -8.177   0.000  0.00  0.00           C+0
HETATM    8  N   UNK     0      20.202  -6.637   0.000  0.00  0.00           N+1
HETATM    9  C   UNK     0      21.742  -6.637   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      18.662  -6.637   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0      20.202  -5.097   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0      14.848 -11.259   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0      16.186 -10.487   0.000  0.00  0.00           C+0
HETATM   14  C   UNK     0      17.523 -11.259   0.000  0.00  0.00           C+0
HETATM   15  C   UNK     0      18.860 -10.487   0.000  0.00  0.00           C+0
HETATM   16  C   UNK     0      20.198 -11.259   0.000  0.00  0.00           C+0
HETATM   17  O   UNK     0      20.198 -12.803   0.000  0.00  0.00           O+0
HETATM   18  O   UNK     0      17.508 -12.745   0.000  0.00  0.00           O+0
CONECT    1    2    5    7                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    6                                                  
CONECT    4    3                                                            
CONECT    5    1   16                                                       
CONECT    6    3                                                            
CONECT    7    1    8                                                       
CONECT    8    7    9   10   11                                             
CONECT    9    8                                                            
CONECT   10    8                                                            
CONECT   11    8                                                            
CONECT   12   13                                                            
CONECT   13   12   14                                                       
CONECT   14   13   15   18                                                  
CONECT   15   14   16                                                       
CONECT   16   15   17    5                                                  
CONECT   17   16                                                            
CONECT   18   14                                                            
MASTER        0    0    0    0    0    0    0    0   18    0   34    0
END

COMPND    HMDB0013132
HETATM    1  C1  UNL     1       4.027  -1.695  -0.298  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.556  -1.319   1.097  1.00  0.00           C  
HETATM    3  C3  UNL     1       2.852   0.014   0.978  1.00  0.00           C  
HETATM    4  O1  UNL     1       2.395   0.413   2.230  1.00  0.00           O  
HETATM    5  C4  UNL     1       1.645  -0.199   0.079  1.00  0.00           C  
HETATM    6  C5  UNL     1       0.944   1.124  -0.040  1.00  0.00           C  
HETATM    7  O2  UNL     1       1.946   1.952  -0.606  1.00  0.00           O  
HETATM    8  O3  UNL     1      -0.146   1.002  -0.770  1.00  0.00           O  
HETATM    9  C6  UNL     1      -1.376   1.343  -0.481  1.00  0.00           C  
HETATM   10  C7  UNL     1      -1.974   2.515  -1.254  1.00  0.00           C  
HETATM   11  C8  UNL     1      -1.185   3.754  -1.040  1.00  0.00           C  
HETATM   12  O4  UNL     1      -0.131   3.970  -1.690  1.00  0.00           O  
HETATM   13  O5  UNL     1      -1.562   4.727  -0.125  1.00  0.00           O1-
HETATM   14  C9  UNL     1      -2.320   0.203  -0.643  1.00  0.00           C  
HETATM   15  N1  UNL     1      -2.175  -0.959   0.125  1.00  0.00           N1+
HETATM   16  C10 UNL     1      -1.421  -1.960  -0.653  1.00  0.00           C  
HETATM   17  C11 UNL     1      -1.616  -0.894   1.421  1.00  0.00           C  
HETATM   18  C12 UNL     1      -3.505  -1.612   0.283  1.00  0.00           C  
HETATM   19  H1  UNL     1       4.235  -0.794  -0.915  1.00  0.00           H  
HETATM   20  H2  UNL     1       4.958  -2.290  -0.273  1.00  0.00           H  
HETATM   21  H3  UNL     1       3.243  -2.271  -0.823  1.00  0.00           H  
HETATM   22  H4  UNL     1       4.368  -1.298   1.826  1.00  0.00           H  
HETATM   23  H5  UNL     1       2.760  -2.059   1.397  1.00  0.00           H  
HETATM   24  H6  UNL     1       3.496   0.797   0.570  1.00  0.00           H  
HETATM   25  H7  UNL     1       3.109   0.730   2.823  1.00  0.00           H  
HETATM   26  H8  UNL     1       1.035  -1.038   0.393  1.00  0.00           H  
HETATM   27  H9  UNL     1       2.080  -0.429  -0.940  1.00  0.00           H  
HETATM   28  H10 UNL     1       0.772   1.525   0.963  1.00  0.00           H  
HETATM   29  H11 UNL     1       1.847   1.896  -1.589  1.00  0.00           H  
HETATM   30  H12 UNL     1      -1.440   1.727   0.573  1.00  0.00           H  
HETATM   31  H13 UNL     1      -2.163   2.297  -2.297  1.00  0.00           H  
HETATM   32  H14 UNL     1      -2.964   2.713  -0.790  1.00  0.00           H  
HETATM   33  H15 UNL     1      -2.375  -0.100  -1.747  1.00  0.00           H  
HETATM   34  H16 UNL     1      -3.397   0.566  -0.497  1.00  0.00           H  
HETATM   35  H17 UNL     1      -2.086  -2.487  -1.343  1.00  0.00           H  
HETATM   36  H18 UNL     1      -0.857  -2.627   0.006  1.00  0.00           H  
HETATM   37  H19 UNL     1      -0.676  -1.367  -1.259  1.00  0.00           H  
HETATM   38  H20 UNL     1      -2.462  -0.919   2.173  1.00  0.00           H  
HETATM   39  H21 UNL     1      -1.104   0.065   1.671  1.00  0.00           H  
HETATM   40  H22 UNL     1      -0.992  -1.772   1.701  1.00  0.00           H  
HETATM   41  H23 UNL     1      -3.905  -1.691  -0.746  1.00  0.00           H  
HETATM   42  H24 UNL     1      -4.134  -0.960   0.925  1.00  0.00           H  
HETATM   43  H25 UNL     1      -3.300  -2.593   0.728  1.00  0.00           H  
CONECT    1    2   19   20   21
CONECT    2    3   22   23
CONECT    3    4    5   24
CONECT    4   25
CONECT    5    6   26   27
CONECT    6    7    8   28
CONECT    7   29
CONECT    8    9
CONECT    9   10   14   30
CONECT   10   11   31   32
CONECT   11   12   12   13
CONECT   14   15   33   34
CONECT   15   16   17   18
CONECT   16   35   36   37
CONECT   17   38   39   40
CONECT   18   41   42   43
END

HMDB0013132
     RDKit          3D
Hydroxyvalerylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    4.0271   -1.6948   -0.2977 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5558   -1.3185    1.0966 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.8518    0.0142    0.9778 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.3948    0.4129    2.2305 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.6449   -0.1992    0.0789 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9444    1.1243   -0.0404 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.9456    1.9521   -0.6059 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1460    1.0024   -0.7701 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3761    1.3430   -0.4811 C   0  0  1  0  0  0  0  0  0  0  0  0
   -1.9738    2.5147   -1.2545 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1851    3.7540   -1.0402 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.1310    3.9699   -1.6901 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.5621    4.7268   -0.1250 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3200    0.2026   -0.6429 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1749   -0.9590    0.1254 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.4207   -1.9604   -0.6528 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6160   -0.8943    1.4206 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5050   -1.6124    0.2829 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2345   -0.7939   -0.9155 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9577   -2.2900   -0.2730 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.2428   -2.2711   -0.8228 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.3679   -1.2984    1.8259 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7599   -2.0587    1.3975 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4962    0.7972    0.5696 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1087    0.7300    2.8227 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.0352   -1.0379    0.3931 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.0797   -0.4293   -0.9401 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.7722    1.5251    0.9635 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.8466    1.8962   -1.5894 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4399    1.7266    0.5732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1629    2.2974   -2.2974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9642    2.7133   -0.7905 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3750   -0.1004   -1.7469 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3968    0.5656   -0.4974 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0860   -2.4869   -1.3427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8574   -2.6269    0.0055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6757   -1.3668   -1.2590 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4617   -0.9192    2.1732 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1040    0.0651    1.6713 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9920   -1.7722    1.7010 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9054   -1.6906   -0.7458 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.1340   -0.9597    0.9250 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3003   -2.5930    0.7278 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  6  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  1  0
 11 12  2  0
 11 13  1  0
  9 14  1  0
 14 15  1  0
 15 16  1  0
 15 17  1  0
 15 18  1  0
  1 19  1  0
  1 20  1  0
  1 21  1  0
  2 22  1  0
  2 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  5 27  1  0
  6 28  1  0
  7 29  1  0
  9 30  1  1
 10 31  1  0
 10 32  1  0
 14 33  1  0
 14 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
 18 41  1  0
 18 42  1  0
 18 43  1  0
M  CHG  2  13  -1  15   1
M  END

View in JSmol View Stereo Labels

Synonyms

Not Available

Chemical Formula

C₁₂H₂₃NO₅

Average Molecular Weight

261.318

Monoisotopic Molecular Weight

261.158

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

Not Available

SMILES

[H]C(O)(CC)CC(=O)O[C@@]([H])(CC([O-])=O)C[N+](C)(C)C

InChI Identifier

InChI=1S/C12H23NO5/c1-5-9(14)6-12(17)18-10(7-11(15)16)8-13(2,3)4/h9-10,14H,5-8H2,1-4H3/t9?,10-/m0/s1

InChI Key

SHDVOJJCEZGPBX-AXDSSHIGSA-N

Chemical Taxonomy

Classification

Not classified

Ontology

Physiological effect

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 24704061)

Excreta

Biofluid or Excreta

Urine (PMID: 26910390)
Feces (PMID: 27624970)

Cell

All cells and tissues (PMID: 35710135)

Cellular substructure

Organelle

Peroxisome (PMID: 35710135)
Adiposome (HMDB: HMDB0013132)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0013132)

Source

Exogenous

Food

Food (HMDB: HMDB0013132)

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

Cow milk, pasteurized, vitamin A + D added, 0% fat (FooDB: FOOD00889)
Cow milk, pasteurized, vitamin A + D added, 1% fat (FooDB: FOOD00890)
Cow milk, pasteurized, vitamin A + D added, 2% fat (FooDB: FOOD00891)
Cow milk, pasteurized, vitamin D added, 3.25% fat (FooDB: FOOD00892)

Endogenous

Animal

Animal (HMDB: HMDB0013132)

Endogenous (HMDB: HMDB0013132)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0013132)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0013132)

Lipid metabolism pathway (HMDB: HMDB0013132)

Biochemical process

Lipid transport (HMDB: HMDB0013132)

Role

Biological role

Energy source (HMDB: HMDB0013132)
Energy source (PMID: 35710135)

Industrial application

Pharmaceutical industry

Biomarker

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	2.28 g/L	ALOGPS

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DeepCCS	[M+H]+	165.621	30932474
DeepCCS	[M-H]-	162.279	30932474
DeepCCS	[M-2H]-	198.019	30932474
DeepCCS	[M+Na]+	174.254	30932474
AllCCS	[M+H]+	164.0	32859911
AllCCS	[M+H-H2O]+	161.0	32859911
AllCCS	[M+NH4]+	166.7	32859911
AllCCS	[M+Na]+	167.5	32859911
AllCCS	[M-H]-	168.9	32859911
AllCCS	[M+Na-2H]-	169.8	32859911
AllCCS	[M+HCOO]-	170.8	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Hydroxyvalerylcarnitine	CCC(O)CC(O)O[C@@H](CC([O-])=O)C[N+](C)(C)C	2772.8	Standard polar	33892256
Hydroxyvalerylcarnitine	CCC(O)CC(O)O[C@@H](CC([O-])=O)C[N+](C)(C)C	1565.9	Standard non polar	33892256
Hydroxyvalerylcarnitine	CCC(O)CC(O)O[C@@H](CC([O-])=O)C[N+](C)(C)C	1849.0	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Hydroxyvalerylcarnitine,1TMS,isomer #1	CCC(CC(O)O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C	1839.4	Semi standard non polar	33892256
Hydroxyvalerylcarnitine,1TMS,isomer #2	CCC(O)CC(O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C	1829.5	Semi standard non polar	33892256
Hydroxyvalerylcarnitine,2TMS,isomer #1	CCC(CC(O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C)O[Si](C)(C)C	1848.6	Semi standard non polar	33892256
Hydroxyvalerylcarnitine,1TBDMS,isomer #1	CCC(CC(O)O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C(C)(C)C	2062.3	Semi standard non polar	33892256
Hydroxyvalerylcarnitine,1TBDMS,isomer #2	CCC(O)CC(O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C(C)(C)C	2039.2	Semi standard non polar	33892256
Hydroxyvalerylcarnitine,2TBDMS,isomer #1	CCC(CC(O[C@@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C(C)(C)C)O[Si](C)(C)C(C)(C)C	2319.4	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 - Hydroxyvalerylcarnitine 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
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 10V, Positive-QTOF	splash10-03di-0190000000-912bc2725d99bd01eec3	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 20V, Positive-QTOF	splash10-000i-9400000000-b7546bc84d9c4708d4d2	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 40V, Positive-QTOF	splash10-000i-9000000000-4508114e92c2ad644e0f	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 10V, Negative-QTOF	splash10-0aor-7900000000-34ed98270bb1cd5138e3	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 20V, Negative-QTOF	splash10-0a4i-9300000000-33707be75f6b0f6886fe	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Hydroxyvalerylcarnitine 40V, Negative-QTOF	splash10-0a4l-9100000000-a64e38da3e0f16f6c83a	2021-09-24	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Not Available

Biospecimen Locations

Blood
Feces
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	0.020-0.100 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Blood	Detected and Quantified	<0.110 uM	Not Specified	Not Specified	Normal	16148061	details
Feces	Detected and Quantified	0.38 +/- 0.24 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	0.2 +/- 0.09 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Urine	Detected and Quantified	143.75 +/- 6.21 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	26910390	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected and Quantified	0.14 (0.10-0.21) umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	Analysis of 40 NI...	details
Urine	Detected and Quantified	0.04-0.29 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	0.06(0.05-0.13) umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	0.08(0.04-0.33) umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.0602 (0.0527) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	0.024 +/- 0.009 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	0.023 +/- 0.007 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Urine	Detected and Quantified	193.30 +/- 6.69 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Obese	26910390	details

Associated Disorders and Diseases

Disease References

Obesity

Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22. [PubMed:26910390 ]
Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.

Associated OMIM IDs

601665 (Obesity)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

Not Available

KNApSAcK ID

Not Available

Chemspider ID

Not Available

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

145453469

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000851

Good Scents ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

Simons K, Toomre D: Lipid rafts and signal transduction. Nat Rev Mol Cell Biol. 2000 Oct;1(1):31-9. [PubMed:11413487 ]
Watson AD: Thematic review series: systems biology approaches to metabolic and cardiovascular disorders. Lipidomics: a global approach to lipid analysis in biological systems. J Lipid Res. 2006 Oct;47(10):2101-11. Epub 2006 Aug 10. [PubMed:16902246 ]
Sethi JK, Vidal-Puig AJ: Thematic review series: adipocyte biology. Adipose tissue function and plasticity orchestrate nutritional adaptation. J Lipid Res. 2007 Jun;48(6):1253-62. Epub 2007 Mar 20. [PubMed:17374880 ]
Lingwood D, Simons K: Lipid rafts as a membrane-organizing principle. Science. 2010 Jan 1;327(5961):46-50. doi: 10.1126/science.1174621. [PubMed:20044567 ]
Violante S, Ijlst L, Ruiter J, Koster J, van Lenthe H, Duran M, de Almeida IT, Wanders RJ, Houten SM, Ventura FV: Substrate specificity of human carnitine acetyltransferase: Implications for fatty acid and branched-chain amino acid metabolism. Biochim Biophys Acta. 2013 Jun;1832(6):773-9. doi: 10.1016/j.bbadis.2013.02.012. Epub 2013 Feb 24. [PubMed:23485643 ]
Grunert SC, Schlatter SM, Schmitt RN, Gemperle-Britschgi C, Mrazova L, Balci MC, Bischof F, Coker M, Das AM, Demirkol M, de Vries M, Gokcay G, Haberle J, Ucar SK, Lotz-Havla AS, Lucke T, Roland D, Rutsch F, Santer R, Schlune A, Staufner C, Schwab KO, Mitchell GA, Sass JO: 3-Hydroxy-3-methylglutaryl-coenzyme A lyase deficiency: Clinical presentation and outcome in a series of 37 patients. Mol Genet Metab. 2017 Jul;121(3):206-215. doi: 10.1016/j.ymgme.2017.05.014. Epub 2017 May 22. [PubMed:28583327 ]
FRITZ IB: Action of carnitine on long chain fatty acid oxidation by liver. Am J Physiol. 1959 Aug;197:297-304. doi: 10.1152/ajplegacy.1959.197.2.297. [PubMed:13825279 ]
Makarova E, Makrecka-Kuka M, Vilks K, Volska K, Sevostjanovs E, Grinberga S, Zarkova-Malkova O, Dambrova M, Liepinsh E: Decreases in Circulating Concentrations of Long-Chain Acylcarnitines and Free Fatty Acids During the Glucose Tolerance Test Represent Tissue-Specific Insulin Sensitivity. Front Endocrinol (Lausanne). 2019 Dec 17;10:870. doi: 10.3389/fendo.2019.00870. eCollection 2019. [PubMed:31920980 ]
Chen C, Hou G, Zeng C, Ren Y, Chen X, Peng C: Metabolomic profiling reveals amino acid and carnitine alterations as metabolic signatures in psoriasis. Theranostics. 2021 Jan 1;11(2):754-767. doi: 10.7150/thno.51154. eCollection 2021. [PubMed:33391503 ]
Donti TR, Blackburn PR, Atwal PS: Holocarboxylase synthetase deficiency pre and post newborn screening. Mol Genet Metab Rep. 2016 Apr 6;7:40-4. doi: 10.1016/j.ymgmr.2016.03.007. eCollection 2016 Jun. [PubMed:27114915 ]
Dambrova M, Makrecka-Kuka M, Kuka J, Vilskersts R, Nordberg D, Attwood MM, Smesny S, Sen ZD, Guo AC, Oler E, Tian S, Zheng J, Wishart DS, Liepinsh E, Schioth HB: Acylcarnitines: Nomenclature, Biomarkers, Therapeutic Potential, Drug Targets, and Clinical Trials. Pharmacol Rev. 2022 Jul;74(3):506-551. doi: 10.1124/pharmrev.121.000408. [PubMed:35710135 ]
Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Showing metabocard for Hydroxyvalerylcarnitine (HMDB0013132)

MOL for HMDB0013132 (Hydroxyvalerylcarnitine)

3D MOL for HMDB0013132 (Hydroxyvalerylcarnitine)

3D SDF for HMDB0013132 (Hydroxyvalerylcarnitine)

3D-SDF for HMDB0013132 (Hydroxyvalerylcarnitine)

PDB for HMDB0013132 (Hydroxyvalerylcarnitine)

3D PDB for HMDB0013132 (Hydroxyvalerylcarnitine)

SMILES for HMDB0013132 (Hydroxyvalerylcarnitine)

INCHI for HMDB0013132 (Hydroxyvalerylcarnitine)

Structure for HMDB0013132 (Hydroxyvalerylcarnitine)

3D Structure for HMDB0013132 (Hydroxyvalerylcarnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra