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Record Information

Version

5.0

Status

Detected but not Quantified

Creation Date

2019-10-02 16:54:10 UTC

Update Date

2022-03-07 03:18:16 UTC

HMDB ID

HMDB0240421

Secondary Accession Numbers

None

Metabolite Identification

Common Name

2-Hydroxyisovalerylcarnitine

Description

2-Hydroxyisovalerylcarnitine belongs to the class of organic compounds known as acylcarnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond. 2-Hydroxyisovalerylcarnitine is an extremely weak basic (i. e. Human blood levels of 2-hydroxyisovalerylcarnitine were reported to be influenced by genetic variants in the gene HLCS which codes for the enzyme holocarboxylase synthetase (PMID: 26401656 ). Holocarboxylase synthetase deficiency (OMIM: 253270 ), also called HLCS deficiency, is a rare inborn error of metabolism (IEM) and autosomal recessive disorder caused by a defective holocarboxylase synthetase. Individuals with HLCS deficiency are unable to utilize biotin effectively.

Structure

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

HMDB0240421
     RDKit          3D
2-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.5207   -1.0765    1.2580 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4068   -0.0587    0.1464 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5765    0.8976    0.2875 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1038    0.7109    0.2345 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.0359    1.6315   -0.7940 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9330   -0.1835    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8180   -1.1150    1.0672 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0932   -0.0646   -0.7162 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2092   -0.9329   -0.7231 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.1382   -1.7499   -2.0035 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1259   -2.5261   -2.0544 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2578   -1.9525   -1.9446 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0620   -3.9039   -2.2228 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.4632   -0.1390   -0.6560 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6520    0.6789    0.4857 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.0552    0.2526    1.7042 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0957    0.8773    0.7075 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1156    2.0216    0.1904 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1870   -0.6047    2.2012 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.9665   -2.0104    1.0511 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6053   -1.3693    1.3794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4909   -0.4994   -0.8615 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4808    1.8055   -0.3318 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6776    1.1102    1.3772 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4851    0.3530   -0.0069 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1320    1.2776    1.1902 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1381    2.0500   -0.9063 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1145   -1.6809    0.0954 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1401   -1.0893   -2.9091 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0015   -2.4258   -2.0668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5967    0.4766   -1.5975 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3157   -0.8710   -0.7062 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9345   -0.8458    1.7423 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7647    0.4769    2.5583 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1361    0.8111    1.9820 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5540    1.1057   -0.2804 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5089   -0.1011    1.0187 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2473    1.6628    1.4486 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1097    2.1989   -0.8992 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6967    2.7986    0.7190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0605    2.0034    0.5328 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
  4  6  1  0
  6  7  2  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
  3 23  1  0
  3 24  1  0
  3 25  1  0
  4 26  1  1
  5 27  1  0
  9 28  1  1
 10 29  1  0
 10 30  1  0
 14 31  1  0
 14 32  1  0
 16 33  1  0
 16 34  1  0
 16 35  1  0
 17 36  1  0
 17 37  1  0
 17 38  1  0
 18 39  1  0
 18 40  1  0
 18 41  1  0
M  CHG  2  13  -1  15   1
M  END


  Mrv1652310021917022D          

 18 17  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  1  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
    0.3572   -1.8563    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.5060   -0.6210    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0690   -0.6177    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
    1.7835   -1.0301    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7800   -1.8550    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0678    0.2072    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  1  0  0  0
 11 12  1  0  0  0  0
 12 13  2  0  0  0  0
 15 16  1  0  0  0  0
 16 14  1  0  0  0  0
 12 15  1  0  0  0  0
 16 17  1  0  0  0  0
 15 18  1  6  0  0  0
M  CHG  2   1   1   7  -1
M  END
> <DATABASE_ID>
HMDB0240421

> <DATABASE_NAME>
hmdb

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

> <INCHI_IDENTIFIER>
InChI=1S/C12H23NO5/c1-8(2)11(16)12(17)18-9(6-10(14)15)7-13(3,4)5/h8-9,11,16H,6-7H2,1-5H3/t9-,11-/m1/s1

> <INCHI_KEY>
FANTUGMIEAASNP-MWLCHTKSSA-N

> <FORMULA>
C12H23NO5

> <MOLECULAR_WEIGHT>
261.318

> <EXACT_MASS>
261.157622845

> <JCHEM_ACCEPTOR_COUNT>
4

> <JCHEM_ATOM_COUNT>
41

> <JCHEM_AVERAGE_POLARIZABILITY>
27.242821609670745

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(3R)-3-{[(2R)-2-hydroxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoate

> <ALOGPS_LOGP>
-2.01

> <JCHEM_LOGP>
-3.807369220138412

> <ALOGPS_LOGS>
-2.95

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA>
12.487621318843967

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.214011381633626

> <JCHEM_PKA_STRONGEST_BASIC>
-3.8978279269968796

> <JCHEM_POLAR_SURFACE_AREA>
86.66000000000001

> <JCHEM_REFRACTIVITY>
87.8285

> <JCHEM_ROTATABLE_BOND_COUNT>
8

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
3.54e-01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
(3R)-3-{[(2R)-2-hydroxy-3-methylbutanoyl]oxy}-4-(trimethylammonio)butanoate

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0240421
     RDKit          3D
2-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.5207   -1.0765    1.2580 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4068   -0.0587    0.1464 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5765    0.8976    0.2875 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1038    0.7109    0.2345 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.0359    1.6315   -0.7940 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9330   -0.1835    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8180   -1.1150    1.0672 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0932   -0.0646   -0.7162 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2092   -0.9329   -0.7231 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.1382   -1.7499   -2.0035 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1259   -2.5261   -2.0544 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2578   -1.9525   -1.9446 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0620   -3.9039   -2.2228 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.4632   -0.1390   -0.6560 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6520    0.6789    0.4857 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.0552    0.2526    1.7042 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0957    0.8773    0.7075 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1156    2.0216    0.1904 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1870   -0.6047    2.2012 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.9665   -2.0104    1.0511 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6053   -1.3693    1.3794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4909   -0.4994   -0.8615 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4808    1.8055   -0.3318 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6776    1.1102    1.3772 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4851    0.3530   -0.0069 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1320    1.2776    1.1902 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1381    2.0500   -0.9063 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1145   -1.6809    0.0954 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1401   -1.0893   -2.9091 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0015   -2.4258   -2.0668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5967    0.4766   -1.5975 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3157   -0.8710   -0.7062 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9345   -0.8458    1.7423 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7647    0.4769    2.5583 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1361    0.8111    1.9820 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5540    1.1057   -0.2804 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5089   -0.1011    1.0187 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2473    1.6628    1.4486 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1097    2.1989   -0.8992 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6967    2.7986    0.7190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0605    2.0034    0.5328 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
  4  6  1  0
  6  7  2  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
  3 23  1  0
  3 24  1  0
  3 25  1  0
  4 26  1  1
  5 27  1  0
  9 28  1  1
 10 29  1  0
 10 30  1  0
 14 31  1  0
 14 32  1  0
 16 33  1  0
 16 34  1  0
 16 35  1  0
 17 36  1  0
 17 37  1  0
 17 38  1  0
 18 39  1  0
 18 40  1  0
 18 41  1  0
M  CHG  2  13  -1  15   1
M  END

HEADER    PROTEIN                                 02-OCT-19   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   02-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  O   UNK     0       0.667  -3.465   0.000  0.00  0.00           O+0
HETATM   14  C   UNK     0       4.678  -1.159   0.000  0.00  0.00           C+0
HETATM   15  C   UNK     0       1.995  -1.153   0.000  0.00  0.00           C+0
HETATM   16  C   UNK     0       3.329  -1.923   0.000  0.00  0.00           C+0
HETATM   17  C   UNK     0       3.323  -3.463   0.000  0.00  0.00           C+0
HETATM   18  O   UNK     0       1.993   0.387   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   15                                                  
CONECT   13   12                                                            
CONECT   14   16                                                            
CONECT   15   16   12   18                                                  
CONECT   16   15   14   17                                                  
CONECT   17   16                                                            
CONECT   18   15                                                            
MASTER        0    0    0    0    0    0    0    0   18    0   34    0
END

COMPND    HMDB0240421
HETATM    1  C1  UNL     1       3.521  -1.076   1.258  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.407  -0.059   0.146  1.00  0.00           C  
HETATM    3  C3  UNL     1       4.576   0.898   0.287  1.00  0.00           C  
HETATM    4  C4  UNL     1       2.104   0.711   0.235  1.00  0.00           C  
HETATM    5  O1  UNL     1       2.036   1.632  -0.794  1.00  0.00           O  
HETATM    6  C5  UNL     1       0.933  -0.183   0.215  1.00  0.00           C  
HETATM    7  O2  UNL     1       0.818  -1.115   1.067  1.00  0.00           O  
HETATM    8  O3  UNL     1      -0.093  -0.065  -0.716  1.00  0.00           O  
HETATM    9  C6  UNL     1      -1.209  -0.933  -0.723  1.00  0.00           C  
HETATM   10  C7  UNL     1      -1.138  -1.750  -2.004  1.00  0.00           C  
HETATM   11  C8  UNL     1       0.126  -2.526  -2.054  1.00  0.00           C  
HETATM   12  O4  UNL     1       1.258  -1.953  -1.945  1.00  0.00           O  
HETATM   13  O5  UNL     1       0.062  -3.904  -2.223  1.00  0.00           O1-
HETATM   14  C9  UNL     1      -2.463  -0.139  -0.656  1.00  0.00           C  
HETATM   15  N1  UNL     1      -2.652   0.679   0.486  1.00  0.00           N1+
HETATM   16  C10 UNL     1      -2.055   0.253   1.704  1.00  0.00           C  
HETATM   17  C11 UNL     1      -4.096   0.877   0.708  1.00  0.00           C  
HETATM   18  C12 UNL     1      -2.116   2.022   0.190  1.00  0.00           C  
HETATM   19  H1  UNL     1       3.187  -0.605   2.201  1.00  0.00           H  
HETATM   20  H2  UNL     1       2.966  -2.010   1.051  1.00  0.00           H  
HETATM   21  H3  UNL     1       4.605  -1.369   1.379  1.00  0.00           H  
HETATM   22  H4  UNL     1       3.491  -0.499  -0.861  1.00  0.00           H  
HETATM   23  H5  UNL     1       4.481   1.805  -0.332  1.00  0.00           H  
HETATM   24  H6  UNL     1       4.678   1.110   1.377  1.00  0.00           H  
HETATM   25  H7  UNL     1       5.485   0.353  -0.007  1.00  0.00           H  
HETATM   26  H8  UNL     1       2.132   1.278   1.190  1.00  0.00           H  
HETATM   27  H9  UNL     1       1.138   2.050  -0.906  1.00  0.00           H  
HETATM   28  H10 UNL     1      -1.114  -1.681   0.095  1.00  0.00           H  
HETATM   29  H11 UNL     1      -1.140  -1.089  -2.909  1.00  0.00           H  
HETATM   30  H12 UNL     1      -2.002  -2.426  -2.067  1.00  0.00           H  
HETATM   31  H13 UNL     1      -2.597   0.477  -1.597  1.00  0.00           H  
HETATM   32  H14 UNL     1      -3.316  -0.871  -0.706  1.00  0.00           H  
HETATM   33  H15 UNL     1      -1.934  -0.846   1.742  1.00  0.00           H  
HETATM   34  H16 UNL     1      -2.765   0.477   2.558  1.00  0.00           H  
HETATM   35  H17 UNL     1      -1.136   0.811   1.982  1.00  0.00           H  
HETATM   36  H18 UNL     1      -4.554   1.106  -0.280  1.00  0.00           H  
HETATM   37  H19 UNL     1      -4.509  -0.101   1.019  1.00  0.00           H  
HETATM   38  H20 UNL     1      -4.247   1.663   1.449  1.00  0.00           H  
HETATM   39  H21 UNL     1      -2.110   2.199  -0.899  1.00  0.00           H  
HETATM   40  H22 UNL     1      -2.697   2.799   0.719  1.00  0.00           H  
HETATM   41  H23 UNL     1      -1.061   2.003   0.533  1.00  0.00           H  
CONECT    1    2   19   20   21
CONECT    2    3    4   22
CONECT    3   23   24   25
CONECT    4    5    6   26
CONECT    5   27
CONECT    6    7    7    8
CONECT    8    9
CONECT    9   10   14   28
CONECT   10   11   29   30
CONECT   11   12   12   13
CONECT   14   15   31   32
CONECT   15   16   17   18
CONECT   16   33   34   35
CONECT   17   36   37   38
CONECT   18   39   40   41
END

HMDB0240421
     RDKit          3D
2-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.5207   -1.0765    1.2580 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4068   -0.0587    0.1464 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.5765    0.8976    0.2875 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1038    0.7109    0.2345 C   0  0  1  0  0  0  0  0  0  0  0  0
    2.0359    1.6315   -0.7940 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.9330   -0.1835    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8180   -1.1150    1.0672 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0932   -0.0646   -0.7162 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2092   -0.9329   -0.7231 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.1382   -1.7499   -2.0035 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.1259   -2.5261   -2.0544 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.2578   -1.9525   -1.9446 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0620   -3.9039   -2.2228 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.4632   -0.1390   -0.6560 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6520    0.6789    0.4857 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.0552    0.2526    1.7042 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0957    0.8773    0.7075 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1156    2.0216    0.1904 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1870   -0.6047    2.2012 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.9665   -2.0104    1.0511 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6053   -1.3693    1.3794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4909   -0.4994   -0.8615 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4808    1.8055   -0.3318 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6776    1.1102    1.3772 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4851    0.3530   -0.0069 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.1320    1.2776    1.1902 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.1381    2.0500   -0.9063 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1145   -1.6809    0.0954 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1401   -1.0893   -2.9091 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0015   -2.4258   -2.0668 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5967    0.4766   -1.5975 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3157   -0.8710   -0.7062 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9345   -0.8458    1.7423 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7647    0.4769    2.5583 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1361    0.8111    1.9820 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5540    1.1057   -0.2804 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5089   -0.1011    1.0187 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2473    1.6628    1.4486 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1097    2.1989   -0.8992 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6967    2.7986    0.7190 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0605    2.0034    0.5328 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
  4  6  1  0
  6  7  2  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
  3 23  1  0
  3 24  1  0
  3 25  1  0
  4 26  1  1
  5 27  1  0
  9 28  1  1
 10 29  1  0
 10 30  1  0
 14 31  1  0
 14 32  1  0
 16 33  1  0
 16 34  1  0
 16 35  1  0
 17 36  1  0
 17 37  1  0
 17 38  1  0
 18 39  1  0
 18 40  1  0
 18 41  1  0
M  CHG  2  13  -1  15   1
M  END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(3R)-3-{[(2R)-2-hydroxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid	HMDB
(2R)-Hydroxyisovalerylcarnitine	HMDB
C5OH+HMG	HMDB
2-Hydroxyisovalerylcarnitine	HMDB

Chemical Formula

C₁₂H₂₃NO₅

Average Molecular Weight

261.318

Monoisotopic Molecular Weight

261.157622845

IUPAC Name

(3R)-3-{[(2R)-2-hydroxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoate

Traditional Name

(3R)-3-{[(2R)-2-hydroxy-3-methylbutanoyl]oxy}-4-(trimethylammonio)butanoate

CAS Registry Number

Not Available

SMILES

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

InChI Identifier

InChI=1S/C12H23NO5/c1-8(2)11(16)12(17)18-9(6-10(14)15)7-13(3,4)5/h8-9,11,16H,6-7H2,1-5H3/t9-,11-/m1/s1

InChI Key

FANTUGMIEAASNP-MWLCHTKSSA-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
Hydroxy fatty acid
Branched fatty acid
Dicarboxylic acid or derivatives
Tetraalkylammonium salt
Quaternary ammonium salt
Secondary alcohol
Carboxylic acid salt
Carboxylic acid ester
Carboxylic acid
Carboxylic acid derivative
Organic nitrogen compound
Organic oxygen compound
Organopnictogen compound
Organic oxide
Hydrocarbon derivative
Organic salt
Organic zwitterion
Organooxygen compound
Organonitrogen compound
Carbonyl group
Amine
Alcohol
Aliphatic acyclic compound

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

Not Available

Ontology

Physiological effect

Not Available

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 26401656)

Process

Not Available

Role

Not Available

Physical Properties

State

Not Available

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
logP	-2	ALOGPS
logP	-3.8	ChemAxon
logS	-3	ALOGPS
pKa (Strongest Acidic)	4.21	ChemAxon
pKa (Strongest Basic)	-3.9	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	4	ChemAxon
Hydrogen Donor Count	1	ChemAxon
Polar Surface Area	86.66 Å²	ChemAxon
Rotatable Bond Count	8	ChemAxon
Refractivity	87.83 m³·mol⁻¹	ChemAxon
Polarizability	27.24 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
DeepCCS	[M+H]+	168.664	30932474
DeepCCS	[M-H]-	164.837	30932474
DeepCCS	[M-2H]-	201.217	30932474
DeepCCS	[M+Na]+	177.227	30932474

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxyisovalerylcarnitine	CC(C)[C@@H](O)C(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	2246.5	Standard polar	33892256
2-Hydroxyisovalerylcarnitine	CC(C)[C@@H](O)C(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1425.7	Standard non polar	33892256
2-Hydroxyisovalerylcarnitine	CC(C)[C@@H](O)C(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1628.7	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
2-Hydroxyisovalerylcarnitine,1TMS,isomer #1	CC(C)[C@@H](O[Si](C)(C)C)C(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C	1677.4	Semi standard non polar	33892256
2-Hydroxyisovalerylcarnitine,1TBDMS,isomer #1	CC(C)[C@@H](O[Si](C)(C)C(C)(C)C)C(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C	1920.4	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 - 2-Hydroxyisovalerylcarnitine 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 - 2-Hydroxyisovalerylcarnitine 10V, Positive-QTOF	splash10-03di-0090000000-2c59ab2896e0b309dfe2	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxyisovalerylcarnitine 20V, Positive-QTOF	splash10-01p9-9050000000-eeb1b523e2b808aebed0	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 2-Hydroxyisovalerylcarnitine 40V, Positive-QTOF	splash10-000i-9000000000-e9262cbaff8cb4ad0ba6	2021-09-25	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-25	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Not Available

Biospecimen Locations

Blood

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)	Not Specified	Normal	26401656	details

Abnormal Concentrations

Not Available

Associated Disorders and Diseases

Disease References

None

Associated OMIM IDs

None

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

Not Available

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

Not Available

Good Scents ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

Burkhardt R, Kirsten H, Beutner F, Holdt LM, Gross A, Teren A, Tonjes A, Becker S, Krohn K, Kovacs P, Stumvoll M, Teupser D, Thiery J, Ceglarek U, Scholz M: Integration of Genome-Wide SNP Data and Gene-Expression Profiles Reveals Six Novel Loci and Regulatory Mechanisms for Amino Acids and Acylcarnitines in Whole Blood. PLoS Genet. 2015 Sep 24;11(9):e1005510. doi: 10.1371/journal.pgen.1005510. eCollection 2015 Sep. [PubMed:26401656 ]

Showing metabocard for 2-Hydroxyisovalerylcarnitine (HMDB0240421)

MOL for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

3D MOL for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

3D SDF for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

3D-SDF for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

PDB for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

3D PDB for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

SMILES for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

INCHI for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

Structure for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

3D Structure for HMDB0240421 (2-Hydroxyisovalerylcarnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra