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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2013-08-28 20:00:53 UTC

Update Date

2021-09-14 15:48:01 UTC

HMDB ID

HMDB0061189

Secondary Accession Numbers

HMDB61189

Metabolite Identification

Common Name

3-Hydroxyisovalerylcarnitine

Description

3-Hydroxyisovalerylcarnitine is structual derivative of 3-hydroxyisovaleric acid and carnitine. Due to its increased concentration in blood, 3-hydroxyisovalerylcarnitine (C5OH-I) is an important indicator for the diagnosis of organic acidemias in newborns (PMID: 18088573 ). Analysis of acylcarnitines by tandem mass spectrometry (MS/MS) has recently been used to screen newborns for organic acidemias and fatty acid oxidation defects (PMID: 7494654 , 9216448 , 11427446 , 12127323 , 14578311 ). These diseases cause the accumulation of acyl-CoA, which is esterified to acylcarnitine by carnitine acyltransferase. Acylcarnitine is then eliminated in the urine, thus acylcarnitine concentration serves as an excellent indicator for these diseases (PMID: 6361812 ). 3-Hydroxyisovalerylcarnitine (C5OH-I) is an indicator for diagnoses of multiple carboxylase deficiency (MCD), 3-methylcrotonyl-CoA carboxylase deficiency and 3-hydroxy-3-methylglutaryl-CoA lyase deficiency (PMID: 18088573 ), which are inborn errors of metabolism.

Structure

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

HMDB0061189
     RDKit          3D
3-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.1569    0.7068    1.8246 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4249    0.2362    0.4342 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9464    0.1335    0.2214 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0011    1.2090   -0.4713 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8676   -1.1297    0.1234 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4043   -1.2303    0.2858 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9233   -1.8129    1.3231 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5128   -0.7287   -0.6171 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9241   -0.8200   -0.4839 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.4758   -1.4148   -1.7157 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9313   -2.7709   -1.9475 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2459   -3.4467   -2.9830 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0590   -3.3969   -1.0801 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3510    0.5995   -0.2112 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7051    0.8744    0.0038 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.8114    1.6651    1.2578 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2571    1.8009   -1.0072 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6404   -0.1876    0.1204 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1192    0.9324    2.3211 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5948   -0.0334    2.4337 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6182    1.6806    1.7948 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4940    0.8072    0.8819 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2667   -0.9268    0.4247 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.1972    0.3031   -0.8665 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7021    1.5092   -1.0946 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3622   -1.8729    0.7978 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1432   -1.3819   -0.9218 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0840   -1.4500    0.4055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5803   -1.5257   -1.7606 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2291   -0.8319   -2.6412 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0009    1.1871   -1.1133 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6940    0.9826    0.6061 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9066    1.8457    1.3651 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2859    2.6160    1.1479 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3941    1.0837    2.0960 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8142    2.7923   -0.7660 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0165    1.4912   -2.0243 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3555    1.8115   -0.8306 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3927    0.1156    0.9177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2645   -1.1473    0.4776 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3161   -0.2752   -0.7862 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
  2  5  1  0
  5  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
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  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


  Mrv1652303072001012D          

 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  0  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
    1.3687   -1.7432    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.1922   -1.7467    0.0000 C   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 18  1  0  0  0  0
 16 17  1  0  0  0  0
M  CHG  2   1   1   7  -1
M  END
> <DATABASE_ID>
HMDB0061189

> <DATABASE_NAME>
hmdb

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

> <INCHI_IDENTIFIER>
InChI=1S/C12H23NO5/c1-12(2,17)7-11(16)18-9(6-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3/t9-/m1/s1

> <INCHI_KEY>
IGLHHSKNBDXCEY-SECBINFHSA-N

> <FORMULA>
C12H23NO5

> <MOLECULAR_WEIGHT>
261.318

> <EXACT_MASS>
261.157622845

> <JCHEM_ACCEPTOR_COUNT>
4

> <JCHEM_ATOM_COUNT>
41

> <JCHEM_AVERAGE_POLARIZABILITY>
27.34498812239227

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

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

> <ALOGPS_LOGP>
-2.12

> <JCHEM_LOGP>
-4.3294642544717465

> <ALOGPS_LOGS>
-3.12

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA>
15.129611459692892

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.2140115583066144

> <JCHEM_PKA_STRONGEST_BASIC>
-2.742108231860251

> <JCHEM_POLAR_SURFACE_AREA>
86.66000000000001

> <JCHEM_REFRACTIVITY>
88.09540000000001

> <JCHEM_ROTATABLE_BOND_COUNT>
8

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.37e-01 g/l

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

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0061189
     RDKit          3D
3-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.1569    0.7068    1.8246 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4249    0.2362    0.4342 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9464    0.1335    0.2214 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0011    1.2090   -0.4713 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8676   -1.1297    0.1234 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4043   -1.2303    0.2858 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9233   -1.8129    1.3231 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5128   -0.7287   -0.6171 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9241   -0.8200   -0.4839 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.4758   -1.4148   -1.7157 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9313   -2.7709   -1.9475 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2459   -3.4467   -2.9830 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0590   -3.3969   -1.0801 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3510    0.5995   -0.2112 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7051    0.8744    0.0038 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.8114    1.6651    1.2578 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2571    1.8009   -1.0072 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6404   -0.1876    0.1204 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1192    0.9324    2.3211 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5948   -0.0334    2.4337 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6182    1.6806    1.7948 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4940    0.8072    0.8819 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2667   -0.9268    0.4247 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.1972    0.3031   -0.8665 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7021    1.5092   -1.0946 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3622   -1.8729    0.7978 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1432   -1.3819   -0.9218 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0840   -1.4500    0.4055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5803   -1.5257   -1.7606 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2291   -0.8319   -2.6412 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0009    1.1871   -1.1133 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6940    0.9826    0.6061 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9066    1.8457    1.3651 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2859    2.6160    1.1479 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3941    1.0837    2.0960 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8142    2.7923   -0.7660 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0165    1.4912   -2.0243 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3555    1.8115   -0.8306 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3927    0.1156    0.9177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2645   -1.1473    0.4776 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3161   -0.2752   -0.7862 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
  2  5  1  0
  5  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
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  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                                 07-MAR-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   07-MAR-20         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  O   UNK     0       2.555  -3.254   0.000  0.00  0.00           O+0
HETATM   18  C   UNK     0       4.092  -3.261   0.000  0.00  0.00           C+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                                                       
CONECT   16   15   14   18   17                                             
CONECT   17   16                                                            
CONECT   18   16                                                            
MASTER        0    0    0    0    0    0    0    0   18    0   34    0
END

COMPND    HMDB0061189
HETATM    1  C1  UNL     1       3.157   0.707   1.825  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.425   0.236   0.434  1.00  0.00           C  
HETATM    3  C3  UNL     1       4.946   0.133   0.221  1.00  0.00           C  
HETATM    4  O1  UNL     1       3.001   1.209  -0.471  1.00  0.00           O  
HETATM    5  C4  UNL     1       2.868  -1.130   0.123  1.00  0.00           C  
HETATM    6  C5  UNL     1       1.404  -1.230   0.286  1.00  0.00           C  
HETATM    7  O2  UNL     1       0.923  -1.813   1.323  1.00  0.00           O  
HETATM    8  O3  UNL     1       0.513  -0.729  -0.617  1.00  0.00           O  
HETATM    9  C6  UNL     1      -0.924  -0.820  -0.484  1.00  0.00           C  
HETATM   10  C7  UNL     1      -1.476  -1.415  -1.716  1.00  0.00           C  
HETATM   11  C8  UNL     1      -0.931  -2.771  -1.947  1.00  0.00           C  
HETATM   12  O4  UNL     1      -1.246  -3.447  -2.983  1.00  0.00           O  
HETATM   13  O5  UNL     1      -0.059  -3.397  -1.080  1.00  0.00           O1-
HETATM   14  C9  UNL     1      -1.351   0.600  -0.211  1.00  0.00           C  
HETATM   15  N1  UNL     1      -2.705   0.874   0.004  1.00  0.00           N1+
HETATM   16  C10 UNL     1      -2.811   1.665   1.258  1.00  0.00           C  
HETATM   17  C11 UNL     1      -3.257   1.801  -1.007  1.00  0.00           C  
HETATM   18  C12 UNL     1      -3.640  -0.188   0.120  1.00  0.00           C  
HETATM   19  H1  UNL     1       4.119   0.932   2.321  1.00  0.00           H  
HETATM   20  H2  UNL     1       2.595  -0.033   2.434  1.00  0.00           H  
HETATM   21  H3  UNL     1       2.618   1.681   1.795  1.00  0.00           H  
HETATM   22  H4  UNL     1       5.494   0.807   0.882  1.00  0.00           H  
HETATM   23  H5  UNL     1       5.267  -0.927   0.425  1.00  0.00           H  
HETATM   24  H6  UNL     1       5.197   0.303  -0.866  1.00  0.00           H  
HETATM   25  H7  UNL     1       3.702   1.509  -1.095  1.00  0.00           H  
HETATM   26  H8  UNL     1       3.362  -1.873   0.798  1.00  0.00           H  
HETATM   27  H9  UNL     1       3.143  -1.382  -0.922  1.00  0.00           H  
HETATM   28  H10 UNL     1      -1.084  -1.450   0.405  1.00  0.00           H  
HETATM   29  H11 UNL     1      -2.580  -1.526  -1.761  1.00  0.00           H  
HETATM   30  H12 UNL     1      -1.229  -0.832  -2.641  1.00  0.00           H  
HETATM   31  H13 UNL     1      -1.001   1.187  -1.113  1.00  0.00           H  
HETATM   32  H14 UNL     1      -0.694   0.983   0.606  1.00  0.00           H  
HETATM   33  H15 UNL     1      -3.907   1.846   1.365  1.00  0.00           H  
HETATM   34  H16 UNL     1      -2.286   2.616   1.148  1.00  0.00           H  
HETATM   35  H17 UNL     1      -2.394   1.084   2.096  1.00  0.00           H  
HETATM   36  H18 UNL     1      -2.814   2.792  -0.766  1.00  0.00           H  
HETATM   37  H19 UNL     1      -3.016   1.491  -2.024  1.00  0.00           H  
HETATM   38  H20 UNL     1      -4.355   1.811  -0.831  1.00  0.00           H  
HETATM   39  H21 UNL     1      -4.393   0.116   0.918  1.00  0.00           H  
HETATM   40  H22 UNL     1      -3.265  -1.147   0.478  1.00  0.00           H  
HETATM   41  H23 UNL     1      -4.316  -0.275  -0.786  1.00  0.00           H  
CONECT    1    2   19   20   21
CONECT    2    3    4    5
CONECT    3   22   23   24
CONECT    4   25
CONECT    5    6   26   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

HMDB0061189
     RDKit          3D
3-Hydroxyisovalerylcarnitine
 41 40  0  0  0  0  0  0  0  0999 V2000
    3.1569    0.7068    1.8246 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4249    0.2362    0.4342 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.9464    0.1335    0.2214 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0011    1.2090   -0.4713 O   0  0  0  0  0  0  0  0  0  0  0  0
    2.8676   -1.1297    0.1234 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4043   -1.2303    0.2858 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9233   -1.8129    1.3231 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.5128   -0.7287   -0.6171 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9241   -0.8200   -0.4839 C   0  0  2  0  0  0  0  0  0  0  0  0
   -1.4758   -1.4148   -1.7157 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9313   -2.7709   -1.9475 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2459   -3.4467   -2.9830 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0590   -3.3969   -1.0801 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.3510    0.5995   -0.2112 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7051    0.8744    0.0038 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.8114    1.6651    1.2578 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2571    1.8009   -1.0072 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6404   -0.1876    0.1204 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1192    0.9324    2.3211 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.5948   -0.0334    2.4337 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6182    1.6806    1.7948 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.4940    0.8072    0.8819 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2667   -0.9268    0.4247 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.1972    0.3031   -0.8665 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7021    1.5092   -1.0946 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3622   -1.8729    0.7978 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.1432   -1.3819   -0.9218 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0840   -1.4500    0.4055 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5803   -1.5257   -1.7606 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2291   -0.8319   -2.6412 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0009    1.1871   -1.1133 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6940    0.9826    0.6061 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9066    1.8457    1.3651 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2859    2.6160    1.1479 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3941    1.0837    2.0960 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8142    2.7923   -0.7660 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0165    1.4912   -2.0243 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3555    1.8115   -0.8306 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3927    0.1156    0.9177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2645   -1.1473    0.4776 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3161   -0.2752   -0.7862 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
  2  5  1  0
  5  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
  3 22  1  0
  3 23  1  0
  3 24  1  0
  4 25  1  0
  5 26  1  0
  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
3-HIVA-carnitine	MeSH
3-Hydroxyisovalerylcarnitine	HMDB

Chemical Formula

C₁₂H₂₃NO₅

Average Molecular Weight

261.318

Monoisotopic Molecular Weight

261.157622845

IUPAC Name

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

Traditional Name

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

CAS Registry Number

99159-87-2

SMILES

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

InChI Identifier

InChI=1S/C12H23NO5/c1-12(2,17)7-11(16)18-9(6-10(14)15)8-13(3,4)5/h9,17H,6-8H2,1-5H3/t9-/m1/s1

InChI Key

IGLHHSKNBDXCEY-SECBINFHSA-N

Chemical Taxonomy

Description

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

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acid esters

Direct Parent

Acyl carnitines

Alternative Parents

Substituents

Acyl-carnitine
Branched fatty acid
Hydroxy fatty acid
Dicarboxylic acid or derivatives
Tetraalkylammonium salt
Tertiary alcohol
Quaternary ammonium salt
Carboxylic acid ester
Carboxylic acid salt
Carboxylic acid derivative
Carboxylic acid
Organic nitrogen compound
Organonitrogen compound
Organooxygen compound
Organic zwitterion
Organic salt
Hydrocarbon derivative
Organic oxide
Organopnictogen compound
Carbonyl group
Organic oxygen compound
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: 18088573)

Excreta

Biofluid or Excreta

Urine (PMID: 18088573)

Source

Exogenous

Exogenous (HMDB: HMDB0061189)

Process

Not Available

Role

Industrial application

Pharmaceutical industry

Biomarker

3 Hydroxy 3 Methylglutaryl Co A Lyase Deficiency (MarkerDB: MDB00029838)

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
Water Solubility	0.28 g/L	ALOGPS
logP	-2.1	ALOGPS
logP	-4.3	ChemAxon
logS	-3.1	ALOGPS
pKa (Strongest Acidic)	4.21	ChemAxon
pKa (Strongest Basic)	-2.7	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	88.1 m³·mol⁻¹	ChemAxon
Polarizability	27.34 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]+	166.155	30932474
DeepCCS	[M-H]-	162.328	30932474
DeepCCS	[M-2H]-	198.641	30932474
DeepCCS	[M+Na]+	174.766	30932474

Predicted Retention Times

Underivatized

Chromatographic Method	Retention Time	Reference
Measured using a Waters Acquity ultraperformance liquid chromatography (UPLC) ethylene-bridged hybrid (BEH) C18 column (100 mm × 2.1 mm; 1.7 μmparticle diameter). Predicted by Afia on May 17, 2022. Predicted by Afia on May 17, 2022.	2.11 minutes	32390414
Predicted by Siyang on May 30, 2022	9.7854 minutes	33406817
Predicted by Siyang using ReTip algorithm on June 8, 2022	6.69 minutes	32390414
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid	802.6 seconds	40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid	191.7 seconds	40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid	108.8 seconds	40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid	152.8 seconds	40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	53.0 seconds	40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid	306.3 seconds	40023050
BfG_NTS_RP1 =Agilent Zorbax Eclipse Plus C18 (2.1 mm x 150 mm, 3.5 um) with Water:ACN and 0.1% Formic Acid	331.3 seconds	40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)	598.7 seconds	40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid	738.7 seconds	40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid	82.8 seconds	40023050
UFZ_Phenomenex = Kinetex Core-Shell C18 2.6 um, 3.0 x 100 mm, Phenomenex with Water:MeOH and 0.1% Formic Acid	1204.2 seconds	40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	190.3 seconds	40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	210.3 seconds	40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate	346.6 seconds	40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA	264.4 seconds	40023050
Meister zic-pHILIC pH9.3 = Merck SeQuant ZIC-pHILIC column with ACN:Water 5mM NH4Ac pH9.3 and 5mM ammonium acetate in water	73.8 seconds	40023050

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
3-Hydroxyisovalerylcarnitine	CC(C)(O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	2340.0	Standard polar	33892256
3-Hydroxyisovalerylcarnitine	CC(C)(O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1338.9	Standard non polar	33892256
3-Hydroxyisovalerylcarnitine	CC(C)(O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1669.9	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
3-Hydroxyisovalerylcarnitine,1TMS,isomer #1	CC(C)(CC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C	1729.0	Semi standard non polar	33892256
3-Hydroxyisovalerylcarnitine,1TBDMS,isomer #1	CC(C)(CC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C)O[Si](C)(C)C(C)(C)C	1972.8	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - 3-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 - 3-Hydroxyisovalerylcarnitine 10V, Positive-QTOF	splash10-03di-0090000000-2c59ab2896e0b309dfe2	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Hydroxyisovalerylcarnitine 20V, Positive-QTOF	splash10-01p9-9050000000-eeb1b523e2b808aebed0	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-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)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Not Available

Biospecimen Locations

Blood
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.51 uM	Adult (>18 years old)	Female	Normal	15505778	details
Blood	Detected and Quantified	0.45 +/- 0.18 uM	Children (1 - 13 years old)	Not Specified	Normal	25557019	details
Blood	Detected and Quantified	0 uM	Children (1 - 13 years old)	Both	Normal	18088573	details
Blood	Detected and Quantified	0.17 (0.13-0.20) uM	Infant (0-1 year old)	Both	Normal	23395542	details
Blood	Detected and Quantified	<0.510 uM	Not Specified	Not Specified	Normal	15505778	details
Blood	Detected and Quantified	0.0000-0.400 uM	Not Specified	Not Specified	Normal	23705938	details
Urine	Detected and Quantified	0.42 umol/mmol creatinine	Children (1-13 years old)	Male	Normal	18088573	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.36 uM	Infant (0-1 year old)	Female	Multiple carboxylase deficiency	18088573	details
Blood	Detected and Quantified	3.56 uM	Children (1-13 years old)	Female	multiple carboxylase deficiency	18088573	details
Blood	Detected and Quantified	5.05 uM	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	15505778	details
Blood	Detected and Quantified	0.12-4.16 uM	Newborn (0-30 days old)	Not Specified	3-Methylcrotonyl-CoA Carboxylase Deficiency	12872837	details
Blood	Detected and Quantified	10100 +/- 4800 uM	Adult (>18 years old)	Both	3-Methylcrotonyl-CoA Carboxylase Deficiency	25732994	details
Blood	Detected and Quantified	4.81 +/- 0.47 uM	Children (1 - 13 years old)	Not Specified	3-hydroxy-3-methylglutaric aciduria	25557019	details
Blood	Detected and Quantified	5.05 uM	Adult (>18 years old)	Female	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	15505778	details
Blood	Detected and Quantified	7.78 uM	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	23705938	details
Urine	Detected and Quantified	18.67 umol/mmol creatinine	Infant (0-1 year old)	Female	Multiple carboxylase deficiency	18088573	details
Urine	Detected and Quantified	80.88 umol/mmol creatinine	Children (1-13 years old)	Female	Multiple carboxylase deficiency	18088573	details
Urine	Detected and Quantified	52.2 +/- 14.7 umol/mmol creatinine	Adult (>18 years old)	Both	3-Methylcrotonyl-CoA Carboxylase Deficiency	25732994	details

Associated Disorders and Diseases

Disease References

Multiple carboxylase deficiency
Maeda Y, Ito T, Ohmi H, Yokoi K, Nakajima Y, Ueta A, Kurono Y, Togari H, Sugiyama N: Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Jul 15;870(2):154-9. Epub 2007 Dec 4. [PubMed:18088573 ]
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Bischof F, Nagele T, Wanders RJ, Trefz FK, Melms A: 3-hydroxy-3-methylglutaryl-CoA lyase deficiency in an adult with leukoencephalopathy. Ann Neurol. 2004 Nov;56(5):727-30. [PubMed:15505778 ] Dos Santos Mello M, Ribas GS, Wayhs CA, Hammerschmidt T, Guerreiro GB, Favenzani JL, Sitta A, de Moura Coelho D, Wajner M, Vargas CR: Increased oxidative stress in patients with 3-hydroxy-3-methylglutaric aciduria. Mol Cell Biochem. 2015 Apr;402(1-2):149-55. doi: 10.1007/s11010-014-2322-x. Epub 2015 Jan 4. [PubMed:25557019 ] Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
3-Methyl-crotonyl-glycinuria
Koeberl DD, Millington DS, Smith WE, Weavil SD, Muenzer J, McCandless SE, Kishnani PS, McDonald MT, Chaing S, Boney A, Moore E, Frazier DM: Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening. J Inherit Metab Dis. 2003;26(1):25-35. [PubMed:12872837 ] Thomsen JA, Lund AM, Olesen JH, Mohr M, Rasmussen J: Is L-Carnitine Supplementation Beneficial in 3-Methylcrotonyl-CoA Carboxylase Deficiency? JIMD Rep. 2015;21:79-88. doi: 10.1007/8904_2014_393. Epub 2015 Mar 3. [PubMed:25732994 ]

Associated OMIM IDs

246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
210200 (3-Methyl-crotonyl-glycinuria)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

Not Available

KNApSAcK ID

Not Available

Chemspider ID

48061039

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

131668011

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

VMH ID

3IVCRN

MarkerDB ID

MDB00029838

Good Scents ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

Nakanishi T, Shimizu A: Identification of 3-hydroxyisovalerylcarnitine in the urine of a patient with multiple carboxylase deficiency. Ann Clin Biochem. 1993 May;30 ( Pt 3):318-20. [PubMed:8517616 ]
van Hove JL, Rutledge SL, Nada MA, Kahler SG, Millington DS: 3-Hydroxyisovalerylcarnitine in 3-methylcrotonyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1995;18(5):592-601. [PubMed:8598640 ]
Bremer J: Carnitine--metabolism and functions. Physiol Rev. 1983 Oct;63(4):1420-80. [PubMed:6361812 ]
Wei J, Fan S, Li L, Xiang X, Zhao R, Wang Z: [Determination of 3-hydroxyisovaleryl carnitine in plasma and urinary excretion by ultra-performance liquid chromatography-tandem mass spectrometry]. Wei Sheng Yan Jiu. 2016 May;45(3):470-4. [PubMed:27459814 ]
Budner M, Surowiec Z, Fudalej P, Hozyasz KK: Whole-blood 3-hydroxyisovalerylcarnitine as a risk factor for orofacial clefts. Arch Oral Biol. 2013 May;58(5):459-61. doi: 10.1016/j.archoralbio.2012.10.013. Epub 2013 Feb 5. [PubMed:23395542 ]
Maeda Y, Ito T, Ohmi H, Yokoi K, Nakajima Y, Ueta A, Kurono Y, Togari H, Sugiyama N: Determination of 3-hydroxyisovalerylcarnitine and other acylcarnitine levels using liquid chromatography-tandem mass spectrometry in serum and urine of a patient with multiple carboxylase deficiency. J Chromatogr B Analyt Technol Biomed Life Sci. 2008 Jul 15;870(2):154-9. Epub 2007 Dec 4. [PubMed:18088573 ]
Rashed MS, Ozand PT, Bucknall MP, Little D: Diagnosis of inborn errors of metabolism from blood spots by acylcarnitines and amino acids profiling using automated electrospray tandem mass spectrometry. Pediatr Res. 1995 Sep;38(3):324-31. [PubMed:7494654 ]
Rashed MS, Bucknall MP, Little D, Awad A, Jacob M, Alamoudi M, Alwattar M, Ozand PT: Screening blood spots for inborn errors of metabolism by electrospray tandem mass spectrometry with a microplate batch process and a computer algorithm for automated flagging of abnormal profiles. Clin Chem. 1997 Jul;43(7):1129-41. [PubMed:9216448 ]
Chace DH, DiPerna JC, Mitchell BL, Sgroi B, Hofman LF, Naylor EW: Electrospray tandem mass spectrometry for analysis of acylcarnitines in dried postmortem blood specimens collected at autopsy from infants with unexplained cause of death. Clin Chem. 2001;47(7):1166-82. [PubMed:11427446 ]
Shigematsu Y, Hirano S, Hata I, Tanaka Y, Sudo M, Sakura N, Tajima T, Yamaguchi S: Newborn mass screening and selective screening using electrospray tandem mass spectrometry in Japan. J Chromatogr B Analyt Technol Biomed Life Sci. 2002 Aug 25;776(1):39-48. [PubMed:12127323 ]
Chace DH, Kalas TA, Naylor EW: Use of tandem mass spectrometry for multianalyte screening of dried blood specimens from newborns. Clin Chem. 2003 Nov;49(11):1797-817. [PubMed:14578311 ]
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 ]
Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Showing metabocard for 3-Hydroxyisovalerylcarnitine (HMDB0061189)

MOL for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

3D MOL for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

3D SDF for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

3D-SDF for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

PDB for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

3D PDB for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

SMILES for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

INCHI for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

Structure for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

3D Structure for HMDB0061189 (3-Hydroxyisovalerylcarnitine)

Predicted Collision Cross Sections

Predicted Retention Times

Underivatized

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra