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Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2022-10-24 19:44:10 UTC

HMDB ID

HMDB0000736

Secondary Accession Numbers

HMDB0002083
HMDB0062556
HMDB0062606
HMDB00736
HMDB02083
HMDB62556
HMDB62606

Metabolite Identification

Common Name

Isobutyryl-L-carnitine

Description

Isobutyryl-L-carnitine is an acylcarnitine. More specifically, it is an isobutyric 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. Isobutyryl-L-carnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine isobutyryl-L-carnitine 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 isobutyryl-L-carnitine is elevated in the blood or plasma of individuals with isobutyryl-coa dehydrogenase deficiency (PMID: 20591710 ), glutaric aciduria type 2 (PMID: 20591710 ), ethylmalonic encephalopathy (PMID: 20591710 ), and gestational diabetes mellitus (PMID: 29626588 ). It is also decreased in the blood or plasma of individuals with traumatic brain injury (PMID: 23560894 ). Isobutyryl-L-carnitine is elevated in the urine of individuals with glutaric aciduria type 2 (PMID: 2288224 ), multiple acyl-CoA dehydrogenation deficiency (PMID: 3383426 ), and acute coronary syndrome (PMID: 30316136 ). 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

HMDB0000736
     RDKit          3D
Isobutyryl-L-carnitine
 38 37  0  0  0  0  0  0  0  0999 V2000
    4.4576   -0.2732   -1.3397 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4623    0.0178   -0.2153 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6326   -0.9105    0.9413 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0758    0.0564   -0.7372 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8572   -0.1373   -1.9562 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0546    0.3071    0.1434 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3292    0.3730   -0.2541 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8062    1.7437    0.1493 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0885    2.8507   -0.4762 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8401    2.6544   -1.2802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4450    4.1521   -0.1717 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0427   -0.7107    0.4979 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4224   -0.8808    0.2688 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.0086   -0.2429   -0.8569 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7336   -2.3145    0.2178 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1468   -0.3870    1.4555 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9902   -0.1163   -2.3196 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7808   -1.3430   -1.2839 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3720    0.3363   -1.1979 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7050    1.0509    0.1439 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6936   -1.2085    0.9903 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3485   -0.3604    1.8794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0304   -1.8282    0.8265 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3241    0.2406   -1.3296 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8936    1.8719   -0.0528 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7183    1.8371    1.2814 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0241    4.6406    0.5857 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8320   -0.5203    1.5877 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5136   -1.6838    0.2727 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7720    0.5221   -0.5875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3110    0.1346   -1.6273 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6178   -1.0178   -1.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7549   -2.4688    0.5888 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6161   -2.6988   -0.8202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0220   -2.8259    0.8800 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6325    0.5327    1.7875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2061   -0.2015    1.1990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0398   -1.1915    2.2271 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  2  0
  4  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  2  0
  9 11  1  0
  7 12  1  0
 12 13  1  0
 13 14  1  0
 13 15  1  0
 13 16  1  0
  1 17  1  0
  1 18  1  0
  1 19  1  0
  2 20  1  0
  3 21  1  0
  3 22  1  0
  3 23  1  0
  7 24  1  6
  8 25  1  0
  8 26  1  0
 11 27  1  0
 12 28  1  0
 12 29  1  0
 14 30  1  0
 14 31  1  0
 14 32  1  0
 15 33  1  0
 15 34  1  0
 15 35  1  0
 16 36  1  0
 16 37  1  0
 16 38  1  0
M  CHG  1  13   1
M  END

  Mrv1652307072020232D          

 16 15  0  0  0  0            999 V2000
   -1.3584   -0.1993    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
   -0.6441   -0.6127    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.0705   -0.1993    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7845   -0.6127    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.4983   -0.1993    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.0705    0.6252    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3584    0.6252    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0726    1.0358    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
   -2.7907    0.6231    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0726    1.8610    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.9259    1.5288    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0665   -0.6230    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0665   -1.4475    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3523   -1.8610    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
   -2.7785   -1.8610    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.7845   -1.4379    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  7  1  6  0  0  0
  1 12  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  6  2  0  0  0  0
  4  5  1  0  0  0  0
  7  8  1  0  0  0  0
  8  9  1  0  0  0  0
  8 10  1  0  0  0  0
  8 11  1  0  0  0  0
 12 13  1  0  0  0  0
 13 15  2  0  0  0  0
 13 14  1  0  0  0  0
  4 16  1  0  0  0  0
M  CHG  2   8   1  14  -1
M  END
> <DATABASE_ID>
HMDB0000736

> <DATABASE_NAME>
hmdb

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

> <INCHI_IDENTIFIER>
InChI=1S/C11H21NO4/c1-8(2)11(15)16-9(6-10(13)14)7-12(3,4)5/h8-9H,6-7H2,1-5H3/t9-/m1/s1

> <INCHI_KEY>
LRCNOZRCYBNMEP-SECBINFHSA-N

> <FORMULA>
C11H21NO4

> <MOLECULAR_WEIGHT>
231.292

> <EXACT_MASS>
231.14705816

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
37

> <JCHEM_AVERAGE_POLARIZABILITY>
24.64310573657653

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
0

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(3R)-3-[(2-methylpropanoyl)oxy]-4-(trimethylazaniumyl)butanoate

> <ALOGPS_LOGP>
-2.13

> <JCHEM_LOGP>
-3.202858512471745

> <ALOGPS_LOGS>
-3.69

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.268923893967359

> <JCHEM_PKA_STRONGEST_BASIC>
-7.089988723429431

> <JCHEM_POLAR_SURFACE_AREA>
66.43

> <JCHEM_REFRACTIVITY>
81.8382

> <JCHEM_ROTATABLE_BOND_COUNT>
7

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
5.79e-02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
(3R)-3-[(2-methylpropanoyl)oxy]-4-(trimethylammonio)butanoate

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000736
     RDKit          3D
Isobutyryl-L-carnitine
 38 37  0  0  0  0  0  0  0  0999 V2000
    4.4576   -0.2732   -1.3397 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4623    0.0178   -0.2153 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6326   -0.9105    0.9413 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0758    0.0564   -0.7372 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8572   -0.1373   -1.9562 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0546    0.3071    0.1434 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3292    0.3730   -0.2541 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8062    1.7437    0.1493 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0885    2.8507   -0.4762 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8401    2.6544   -1.2802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4450    4.1521   -0.1717 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0427   -0.7107    0.4979 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4224   -0.8808    0.2688 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.0086   -0.2429   -0.8569 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7336   -2.3145    0.2178 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1468   -0.3870    1.4555 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9902   -0.1163   -2.3196 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7808   -1.3430   -1.2839 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3720    0.3363   -1.1979 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7050    1.0509    0.1439 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6936   -1.2085    0.9903 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3485   -0.3604    1.8794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0304   -1.8282    0.8265 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3241    0.2406   -1.3296 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8936    1.8719   -0.0528 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7183    1.8371    1.2814 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0241    4.6406    0.5857 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8320   -0.5203    1.5877 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5136   -1.6838    0.2727 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7720    0.5221   -0.5875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3110    0.1346   -1.6273 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6178   -1.0178   -1.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7549   -2.4688    0.5888 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6161   -2.6988   -0.8202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0220   -2.8259    0.8800 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6325    0.5327    1.7875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2061   -0.2015    1.1990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0398   -1.1915    2.2271 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  2  0
  4  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  2  0
  9 11  1  0
  7 12  1  0
 12 13  1  0
 13 14  1  0
 13 15  1  0
 13 16  1  0
  1 17  1  0
  1 18  1  0
  1 19  1  0
  2 20  1  0
  3 21  1  0
  3 22  1  0
  3 23  1  0
  7 24  1  6
  8 25  1  0
  8 26  1  0
 11 27  1  0
 12 28  1  0
 12 29  1  0
 14 30  1  0
 14 31  1  0
 14 32  1  0
 15 33  1  0
 15 34  1  0
 15 35  1  0
 16 36  1  0
 16 37  1  0
 16 38  1  0
M  CHG  1  13   1
M  END

HEADER    PROTEIN                                 07-JUL-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   07-JUL-20         0                                  
HETATM    1  C   UNK     0      -2.536  -0.372   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0      -1.202  -1.144   0.000  0.00  0.00           O+0
HETATM    3  C   UNK     0       0.132  -0.372   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0       1.464  -1.144   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0       2.797  -0.372   0.000  0.00  0.00           C+0
HETATM    6  O   UNK     0       0.132   1.167   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0      -2.536   1.167   0.000  0.00  0.00           C+0
HETATM    8  N   UNK     0      -3.869   1.934   0.000  0.00  0.00           N+1
HETATM    9  C   UNK     0      -5.209   1.163   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      -3.869   3.474   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0      -5.462   2.854   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0      -3.857  -1.163   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0      -3.857  -2.702   0.000  0.00  0.00           C+0
HETATM   14  O   UNK     0      -2.524  -3.474   0.000  0.00  0.00           O-1
HETATM   15  O   UNK     0      -5.187  -3.474   0.000  0.00  0.00           O+0
HETATM   16  C   UNK     0       1.464  -2.684   0.000  0.00  0.00           C+0
CONECT    1    2    7   12                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    6                                                  
CONECT    4    3    5   16                                                  
CONECT    5    4                                                            
CONECT    6    3                                                            
CONECT    7    1    8                                                       
CONECT    8    7    9   10   11                                             
CONECT    9    8                                                            
CONECT   10    8                                                            
CONECT   11    8                                                            
CONECT   12    1   13                                                       
CONECT   13   12   15   14                                                  
CONECT   14   13                                                            
CONECT   15   13                                                            
CONECT   16    4                                                            
MASTER        0    0    0    0    0    0    0    0   16    0   30    0
END

COMPND    HMDB0000736
HETATM    1  C1  UNL     1       4.458  -0.273  -1.340  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.462   0.018  -0.215  1.00  0.00           C  
HETATM    3  C3  UNL     1       3.633  -0.911   0.941  1.00  0.00           C  
HETATM    4  C4  UNL     1       2.076   0.056  -0.737  1.00  0.00           C  
HETATM    5  O1  UNL     1       1.857  -0.137  -1.956  1.00  0.00           O  
HETATM    6  O2  UNL     1       1.055   0.307   0.143  1.00  0.00           O  
HETATM    7  C5  UNL     1      -0.329   0.373  -0.254  1.00  0.00           C  
HETATM    8  C6  UNL     1      -0.806   1.744   0.149  1.00  0.00           C  
HETATM    9  C7  UNL     1      -0.089   2.851  -0.476  1.00  0.00           C  
HETATM   10  O3  UNL     1       0.840   2.654  -1.280  1.00  0.00           O  
HETATM   11  O4  UNL     1      -0.445   4.152  -0.172  1.00  0.00           O  
HETATM   12  C8  UNL     1      -1.043  -0.711   0.498  1.00  0.00           C  
HETATM   13  N1  UNL     1      -2.422  -0.881   0.269  1.00  0.00           N1+
HETATM   14  C9  UNL     1      -3.009  -0.243  -0.857  1.00  0.00           C  
HETATM   15  C10 UNL     1      -2.734  -2.315   0.218  1.00  0.00           C  
HETATM   16  C11 UNL     1      -3.147  -0.387   1.455  1.00  0.00           C  
HETATM   17  H1  UNL     1       3.990  -0.116  -2.320  1.00  0.00           H  
HETATM   18  H2  UNL     1       4.781  -1.343  -1.284  1.00  0.00           H  
HETATM   19  H3  UNL     1       5.372   0.336  -1.198  1.00  0.00           H  
HETATM   20  H4  UNL     1       3.705   1.051   0.144  1.00  0.00           H  
HETATM   21  H5  UNL     1       4.694  -1.209   0.990  1.00  0.00           H  
HETATM   22  H6  UNL     1       3.349  -0.360   1.879  1.00  0.00           H  
HETATM   23  H7  UNL     1       3.030  -1.828   0.826  1.00  0.00           H  
HETATM   24  H8  UNL     1      -0.324   0.241  -1.330  1.00  0.00           H  
HETATM   25  H9  UNL     1      -1.894   1.872  -0.053  1.00  0.00           H  
HETATM   26  H10 UNL     1      -0.718   1.837   1.281  1.00  0.00           H  
HETATM   27  H11 UNL     1      -0.024   4.641   0.586  1.00  0.00           H  
HETATM   28  H12 UNL     1      -0.832  -0.520   1.588  1.00  0.00           H  
HETATM   29  H13 UNL     1      -0.514  -1.684   0.273  1.00  0.00           H  
HETATM   30  H14 UNL     1      -3.772   0.522  -0.588  1.00  0.00           H  
HETATM   31  H15 UNL     1      -2.311   0.135  -1.627  1.00  0.00           H  
HETATM   32  H16 UNL     1      -3.618  -1.018  -1.418  1.00  0.00           H  
HETATM   33  H17 UNL     1      -3.755  -2.469   0.589  1.00  0.00           H  
HETATM   34  H18 UNL     1      -2.616  -2.699  -0.820  1.00  0.00           H  
HETATM   35  H19 UNL     1      -2.022  -2.826   0.880  1.00  0.00           H  
HETATM   36  H20 UNL     1      -2.632   0.533   1.788  1.00  0.00           H  
HETATM   37  H21 UNL     1      -4.206  -0.202   1.199  1.00  0.00           H  
HETATM   38  H22 UNL     1      -3.040  -1.192   2.227  1.00  0.00           H  
CONECT    1    2   17   18   19
CONECT    2    3    4   20
CONECT    3   21   22   23
CONECT    4    5    5    6
CONECT    6    7
CONECT    7    8   12   24
CONECT    8    9   25   26
CONECT    9   10   10   11
CONECT   11   27
CONECT   12   13   28   29
CONECT   13   14   15   16
CONECT   14   30   31   32
CONECT   15   33   34   35
CONECT   16   36   37   38
END

HMDB0000736
     RDKit          3D
Isobutyryl-L-carnitine
 38 37  0  0  0  0  0  0  0  0999 V2000
    4.4576   -0.2732   -1.3397 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.4623    0.0178   -0.2153 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.6326   -0.9105    0.9413 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0758    0.0564   -0.7372 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8572   -0.1373   -1.9562 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0546    0.3071    0.1434 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3292    0.3730   -0.2541 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8062    1.7437    0.1493 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0885    2.8507   -0.4762 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8401    2.6544   -1.2802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4450    4.1521   -0.1717 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0427   -0.7107    0.4979 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4224   -0.8808    0.2688 N   0  0  0  0  0  4  0  0  0  0  0  0
   -3.0086   -0.2429   -0.8569 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.7336   -2.3145    0.2178 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1468   -0.3870    1.4555 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9902   -0.1163   -2.3196 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7808   -1.3430   -1.2839 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.3720    0.3363   -1.1979 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7050    1.0509    0.1439 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.6936   -1.2085    0.9903 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3485   -0.3604    1.8794 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.0304   -1.8282    0.8265 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3241    0.2406   -1.3296 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8936    1.8719   -0.0528 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7183    1.8371    1.2814 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0241    4.6406    0.5857 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8320   -0.5203    1.5877 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5136   -1.6838    0.2727 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7720    0.5221   -0.5875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.3110    0.1346   -1.6273 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6178   -1.0178   -1.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.7549   -2.4688    0.5888 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6161   -2.6988   -0.8202 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.0220   -2.8259    0.8800 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6325    0.5327    1.7875 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2061   -0.2015    1.1990 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0398   -1.1915    2.2271 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  2  0
  4  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  2  0
  9 11  1  0
  7 12  1  0
 12 13  1  0
 13 14  1  0
 13 15  1  0
 13 16  1  0
  1 17  1  0
  1 18  1  0
  1 19  1  0
  2 20  1  0
  3 21  1  0
  3 22  1  0
  3 23  1  0
  7 24  1  6
  8 25  1  0
  8 26  1  0
 11 27  1  0
 12 28  1  0
 12 29  1  0
 14 30  1  0
 14 31  1  0
 14 32  1  0
 15 33  1  0
 15 34  1  0
 15 35  1  0
 16 36  1  0
 16 37  1  0
 16 38  1  0
M  CHG  1  13   1
M  END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(R)-Isobutyrylcarnitine	ChEBI
Iso-butyryl-L(-)-carnitin	ChEBI
Isobutyryl-carnitine	ChEBI
Isobutyryl-L-(-)-carnitine	ChEBI
O-Isobutanoyl-(R)-carnitine	ChEBI
Isobutyryl-1-carnitine	HMDB
Isobutyrylcarnitine	HMDB
O-Isobutyryl-L-carnitine	HMDB
Isobutyryl-L-carnitine	HMDB, ChEBI

Chemical Formula

C₁₁H₂₂NO₄

Average Molecular Weight

232.299

Monoisotopic Molecular Weight

232.154334613

IUPAC Name

(3R)-3-[(2-methylpropanoyl)oxy]-4-(trimethylazaniumyl)butanoate

Traditional Name

(3R)-3-[(2-methylpropanoyl)oxy]-4-(trimethylammonio)butanoate

CAS Registry Number

25518-49-4

SMILES

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

InChI Identifier

InChI=1S/C11H21NO4/c1-8(2)11(15)16-9(6-10(13)14)7-12(3,4)5/h8-9H,6-7H2,1-5H3/p+1/t9-/m1/s1

InChI Key

LRCNOZRCYBNMEP-SECBINFHSA-O

Chemical Taxonomy

Description

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

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acid esters

Direct Parent

Acyl carnitines

Alternative Parents

Substituents

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

O-acyl-L-carnitine (CHEBI:84838 )
O-isobutyrylcarnitine (CHEBI:84838 )

Ontology

Physiological effect

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 9034211)

Organ

Placenta (HMDB: HMDB0000736)

Excreta

Biofluid or Excreta

Urine (PMID: 7776094)

Cellular substructure

Organelle

Peroxisome (PMID: 35710135)

Cell

All cells and tissues (PMID: 35710135)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000736)

Source

Endogenous

Endogenous (HMDB: HMDB0000736)

Animal

Animal (HMDB: HMDB0000736)

Exogenous

Food

Food (HMDB: HMDB0000736)

Animal origin

Poultry

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

Lagomorph

Ovis

Sheep (Mutton, Lamb) (FooDB: FOOD00473)

Bovine

Venison

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

Caprae

Domestic goat (FooDB: FOOD00529)

Swine

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

Equine

Horse (FooDB: FOOD00378)

Milk and milk product

Unfermented milk

Milk (Cow) (FooDB: FOOD00618)

Exogenous (HMDB: HMDB0000736)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0000736)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0000736)

Lipid metabolism pathway (HMDB: HMDB0000736)

Biochemical process

Lipid transport (HMDB: HMDB0000736)

Role

Biological role

Energy source (HMDB: HMDB0000736)
Energy source (PMID: 35710135)

Industrial application

Pharmaceutical industry

Biomarker

Breast cancer ()
Diabetes mellitus type 2 ()
Cardiovascular disease ()
Renal cell carcinoma ()
Heart failure ()
Celiac disease ()
Inflammatory bowel disease ()
Short Chain Acyl-CoA Dehydrogenase Deficiency (MarkerDB: MDB00000241)

Physical Properties

State

Solid

Experimental Molecular Properties

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

Experimental Chromatographic Properties

Not Available

Predicted Molecular Properties

Property	Value	Source
Water Solubility	0.058 g/L	ALOGPS
logP	-2.1	ALOGPS
logP	-3.2	ChemAxon
logS	-3.7	ALOGPS
pKa (Strongest Acidic)	4.27	ChemAxon
pKa (Strongest Basic)	-7.1	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	0	ChemAxon
Polar Surface Area	66.43 Å²	ChemAxon
Rotatable Bond Count	7	ChemAxon
Refractivity	81.84 m³·mol⁻¹	ChemAxon
Polarizability	24.64 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]+	153.648	30932474
DeepCCS	[M-H]-	151.289	30932474
DeepCCS	[M-2H]-	184.173	30932474
DeepCCS	[M+Na]+	159.741	30932474
AllCCS	[M+H]+	153.7	32859911
AllCCS	[M+H-H2O]+	150.6	32859911
AllCCS	[M+NH4]+	156.5	32859911
AllCCS	[M+Na]+	157.3	32859911
AllCCS	[M-H]-	161.7	32859911
AllCCS	[M+Na-2H]-	162.9	32859911
AllCCS	[M+HCOO]-	164.3	32859911

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Isobutyryl-L-carnitine	CC(C)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	2355.0	Standard polar	33892256
Isobutyryl-L-carnitine	CC(C)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	1387.9	Standard non polar	33892256
Isobutyryl-L-carnitine	CC(C)C(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	1558.5	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Isobutyryl-L-carnitine,1TMS,isomer #1	CC(C)C(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C	1548.9	Semi standard non polar	33892256
Isobutyryl-L-carnitine,1TBDMS,isomer #1	CC(C)C(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C	1775.5	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 - Isobutyryl-L-carnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyryl-L-carnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyryl-L-carnitine GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Isobutyryl-L-carnitine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyryl-L-carnitine Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-001i-5290000000-baab610a0cdc91406add	2018-05-25	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyryl-L-carnitine Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-001i-9000000000-8d180efb75e39baa17aa	2018-05-25	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Isobutyryl-L-carnitine Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-001i-9000000000-6311075865704b40d294	2018-05-25	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyryl-L-carnitine 10V, Positive-QTOF	splash10-001i-0090000000-cf95ec9e05e8b6c1a98f	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyryl-L-carnitine 20V, Positive-QTOF	splash10-0019-9050000000-ebf3a8e1eb1d0ceaaeba	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Isobutyryl-L-carnitine 40V, Positive-QTOF	splash10-000i-9000000000-e9262cbaff8cb4ad0ba6	2021-09-22	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-24	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
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, H₂O, experimental)	2018-05-25	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Extracellular
Membrane

Biospecimen Locations

Blood
Urine

Tissue Locations

Placenta

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.069 (0.015-0.203) uM	Children (1 - 13 years old)	Both	Normal	9034211	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	0.069 (0.015-0.203) uM	Children (1-13 years old)	Both	Normal	9034211	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Infant (0-1 year old)	Both	Normal	7776094	details
Urine	Detected and Quantified	0.05-0.33 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	0.11 +/- 0.06 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	0.13 +/- 0.07 umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	32340350	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.034 (0.009-0.084) uM	Adult (>18 years old)	Both	Very long-chain acyl-CoA dehydrogenase deficiency (vLCAD)	9034211	details
Urine	Detected and Quantified	0 umol/mmol creatinine	Infant (0-1 year old)	Both	Short chain acyl-CoA dehydrogenase deficiency (SCAD)	7776094	details

Associated Disorders and Diseases

Disease References

Very Long Chain Acyl-CoA Dehydrogenase Deficiency
Costa CG, Struys EA, Bootsma A, ten Brink HJ, Dorland L, Tavares de Almeida I, Duran M, Jakobs C: Quantitative analysis of plasma acylcarnitines using gas chromatography chemical ionization mass fragmentography. J Lipid Res. 1997 Jan;38(1):173-82. [PubMed:9034211 ]
Short Chain Acyl-Coa Dehydrogenase Deficiency
Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [PubMed:7776094 ]

Associated OMIM IDs

201475 (Very Long Chain Acyl-CoA Dehydrogenase Deficiency)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022213

KNApSAcK ID

Not Available

Chemspider ID

147286

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5704

PubChem Compound

168379

PDB ID

Not Available

ChEBI ID

84838

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

MDB00000241

Good Scents ID

Not Available

References

Synthesis Reference

Strack, Erich; Mueller, Detlef M. Preparation of O-acylcarnitines. Hoppe-Seyler's Zeitschrift fuer Physiologische Chemie (1970), 351(1), 95-8.

Material Safety Data Sheet (MSDS)

Not Available

General References

Sass JO, Sander S, Zschocke J: Isobutyryl-CoA dehydrogenase deficiency: isobutyrylglycinuria and ACAD8 gene mutations in two infants. J Inherit Metab Dis. 2004;27(6):741-5. [PubMed:15505379 ]
Kidouchi K, Niwa T, Nohara D, Asai K, Sugiyama N, Morishita H, Kobayashi M, Wada Y: Urinary acylcarnitines in a patient with neonatal multiple acyl-CoA dehydrogenation deficiency, quantified by a carboxylic acid analyzer with a reversed-phase column. Clin Chim Acta. 1988 Apr 29;173(3):263-72. [PubMed:3383426 ]
Minkler PE, Ingalls ST, Hoppel CL: High-performance liquid chromatographic separation of acylcarnitines following derivatization with 4'-bromophenacyl trifluoromethanesulfonate. Anal Biochem. 1990 Feb 15;185(1):29-35. [PubMed:2344045 ]
Roe CR, Cederbaum SD, Roe DS, Mardach R, Galindo A, Sweetman L: Isolated isobutyryl-CoA dehydrogenase deficiency: an unrecognized defect in human valine metabolism. Mol Genet Metab. 1998 Dec;65(4):264-71. [PubMed:9889013 ]
Sakuma T, Sugiyama N, Ichiki T, Kobayashi M, Wada Y, Nohara D: Analysis of acylcarnitines in maternal urine for prenatal diagnosis of glutaric aciduria type 2. Prenat Diagn. 1991 Feb;11(2):77-82. [PubMed:2062823 ]
Vallee L, Fontaine M, Nuyts JP, Ricart G, Krivosic I, Divry P, Vianey-Saban C, Lhermitte M, Vamecq J: Stroke, hemiparesis and deficient mitochondrial beta-oxidation. Eur J Pediatr. 1994 Aug;153(8):598-603. [PubMed:7957409 ]
Sugiyama N, Kidouchi K, Kobayashi M, Wada Y: Carnitine deficiency in inherited organic acid disorders and Reye syndrome. Acta Paediatr Jpn. 1990 Aug;32(4):410-6. [PubMed:2288224 ]
Koeberl DD, Young SP, Gregersen NS, Vockley J, Smith WE, Benjamin DK Jr, An Y, Weavil SD, Chaing SH, Bali D, McDonald MT, Kishnani PS, Chen YT, Millington DS: Rare disorders of metabolism with elevated butyryl- and isobutyryl-carnitine detected by tandem mass spectrometry newborn screening. Pediatr Res. 2003 Aug;54(2):219-23. Epub 2003 May 7. [PubMed:12736383 ]
Fontaine M, Briand G, Largilliere C, Degand P, Divry P, Vianey-Saban C, Mousson B, Vamecq J: Metabolic studies in a patient with severe carnitine palmitoyltransferase type II deficiency. Clin Chim Acta. 1998 May 25;273(2):161-70. [PubMed:9657346 ]
Matsumoto K, Takahashi M, Takiyama N, Misaki H, Matsuo N, Murano S, Yuki H: Enzyme reactor for urinary acylcarnitines assay by reversed-phase high-performance liquid chromatography. Clin Chim Acta. 1993 Jul 16;216(1-2):135-43. [PubMed:8222264 ]
Shalev DP, Soffer Y, Lewin LM: Investigations on the motility of human spermatozoa in a defined medium in the presence of metabolic inhibitors and of carnitine. Andrologia. 1986 Jul-Aug;18(4):368-75. [PubMed:3752540 ]
Fontaine M, Briand G, Ser N, Armelin I, Rolland MO, Degand P, Vamecq J: Metabolic studies in twin brothers with 2-methylacetoacetyl-CoA thiolase deficiency. Clin Chim Acta. 1996 Nov 15;255(1):67-83. [PubMed:8930414 ]
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 ]
Elshenawy S, Pinney SE, Stuart T, Doulias PT, Zura G, Parry S, Elovitz MA, Bennett MJ, Bansal A, Strauss JF 3rd, Ischiropoulos H, Simmons RA: The Metabolomic Signature of the Placenta in Spontaneous Preterm Birth. Int J Mol Sci. 2020 Feb 4;21(3). pii: ijms21031043. doi: 10.3390/ijms21031043. [PubMed:32033212 ]
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 ]
Forni S, Fu X, Palmer SE, Sweetman L: Rapid determination of C4-acylcarnitine and C5-acylcarnitine isomers in plasma and dried blood spots by UPLC-MS/MS as a second tier test following flow-injection MS/MS acylcarnitine profile analysis. Mol Genet Metab. 2010 Sep;101(1):25-32. doi: 10.1016/j.ymgme.2010.05.012. Epub 2010 Jun 10. [PubMed:20591710 ]
Roy C, Tremblay PY, Anassour-Laouan-Sidi E, Lucas M, Forest JC, Giguere Y, Ayotte P: Risk of gestational diabetes mellitus in relation to plasma concentrations of amino acids and acylcarnitines: A nested case-control study. Diabetes Res Clin Pract. 2018 Jun;140:183-190. doi: 10.1016/j.diabres.2018.03.058. Epub 2018 Apr 4. [PubMed:29626588 ]
Jeter CB, Hergenroeder GW, Ward NH 3rd, Moore AN, Dash PK: Human mild traumatic brain injury decreases circulating branched-chain amino acids and their metabolite levels. J Neurotrauma. 2013 Apr 15;30(8):671-9. doi: 10.1089/neu.2012.2491. Epub 2013 Apr 6. [PubMed:23560894 ]
Wang Y, Sun W, Zheng J, Xu C, Wang X, Li T, Tang Y, Li Z: Urinary metabonomic study of patients with acute coronary syndrome using UPLC-QTOF/MS. J Chromatogr B Analyt Technol Biomed Life Sci. 2018 Nov 15;1100-1101:122-130. doi: 10.1016/j.jchromb.2018.10.005. Epub 2018 Oct 7. [PubMed:30316136 ]
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 Isobutyryl-L-carnitine (HMDB0000736)

MOL for HMDB0000736 (Isobutyryl-L-carnitine)

3D MOL for HMDB0000736 (Isobutyryl-L-carnitine)

3D SDF for HMDB0000736 (Isobutyryl-L-carnitine)

3D-SDF for HMDB0000736 (Isobutyryl-L-carnitine)

PDB for HMDB0000736 (Isobutyryl-L-carnitine)

3D PDB for HMDB0000736 (Isobutyryl-L-carnitine)

SMILES for HMDB0000736 (Isobutyryl-L-carnitine)

INCHI for HMDB0000736 (Isobutyryl-L-carnitine)

Structure for HMDB0000736 (Isobutyryl-L-carnitine)

3D Structure for HMDB0000736 (Isobutyryl-L-carnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra