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Record Information

Version

5.0

Status

Detected but not Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2022-10-24 19:27:59 UTC

HMDB ID

HMDB0000378

Secondary Accession Numbers

HMDB0001963
HMDB00378
HMDB01963

Metabolite Identification

Common Name

2-Methylbutyroylcarnitine

Description

2-Methylbutyroylcarnitine is an acylcarnitine. More specifically, it is an 2-methylbutanoic 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. 2-Methylbutyroylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine 2-methylbutyroylcarnitine 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 2-methylbutyroylcarnitine is elevated in the blood or plasma of individuals with exudative age-related macular degeneration (PMID: 32120889 ), type 2 Diabetes Mellitus (PMID: 31782507 , PMID: 20111019 ), obesity (PMID: 20111019 ), acute cerebral infarction (PMID: 29265114 ), diastolic heart failure (PMID: 26010610 ), systolic heart failure (PMID: 26010610 ). It is also decreased in the blood or plasma of individuals with pregnancy (PMID: 24704061 - in serum of pregnant women with fetus with CHD). 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

HMDB0000378
     RDKit          3D
2-Methylbutyroylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    3.8272    2.3739   -0.0034 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6710    1.4507   -0.3578 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.7721    0.2427    0.5122 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1237   -0.4077    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6665   -0.7293    0.3328 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8822   -1.9273   -0.0371 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.3415   -0.4549    0.5380 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7193   -1.3863    0.3675 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4271   -1.7139    1.6204 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5203   -2.6932    1.4567 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8729   -3.2749    0.4249 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2788   -3.0303    2.6033 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.5506   -0.9783   -0.8085 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1799    0.2800   -0.7574 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.3733    1.3459   -0.2161 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4269    0.6809   -2.1587 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5022    0.2508   -0.1340 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5485    3.4299   -0.2057 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7214    2.1636   -0.6384 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0502    2.3182    1.0816 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7375    2.0190   -0.0800 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6395    1.2424   -1.4253 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7750    0.5457    1.5727 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4088   -0.2810   -0.8509 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9137    0.0193    0.8467 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0860   -1.5064    0.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2067   -2.3151    0.0484 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6761   -2.2258    2.2948 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8640   -0.8440    2.1512 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2423   -1.7522   -1.1267 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7973   -0.9177   -1.6617 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4422    1.3934   -0.8424 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1843    1.2836    0.8521 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8602    2.3465   -0.3909 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1504    1.5099   -2.1849 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4376    1.0054   -2.5427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8321   -0.1438   -2.7520 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9443   -0.7705   -0.3283 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4731    0.4934    0.9351 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2028    0.9572   -0.6202 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  2 22  1  0
  3 23  1  0
  4 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

HMDB00378.mol
  Mrv1652305261923542D          

 17 16  0  0  0  0            999 V2000
   -1.0717    0.4125    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
   -0.3572    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572    0.4125    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572    1.2375    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717    0.0000    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717   -0.8250    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -1.2375    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.3572   -2.0625    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572   -2.4750    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.3572   -0.8250    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862   -1.2375    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7862    0.0000    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6020    1.0445    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5414    1.0445    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.0717    1.6500    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.7862    1.2375    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
    1.0717    2.4750    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
  3  5  1  0  0  0  0
  5  6  1  0  0  0  0
  6  7  1  0  0  0  0
  7  8  1  0  0  0  0
  8  9  1  0  0  0  0
  7 10  1  0  0  0  0
  6 11  2  0  0  0  0
  1 12  1  0  0  0  0
  1 13  1  0  0  0  0
  1 14  1  0  0  0  0
  4 15  1  0  0  0  0
 15 16  1  0  0  0  0
 15 17  2  0  0  0  0
M  CHG  2   1   1  16  -1
M  END
> <DATABASE_ID>
HMDB0000378

> <DATABASE_NAME>
hmdb

> <SMILES>
CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C

> <INCHI_IDENTIFIER>
InChI=1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3

> <INCHI_KEY>
IHCPDBBYTYJYIL-UHFFFAOYSA-N

> <FORMULA>
C12H23NO4

> <MOLECULAR_WEIGHT>
245.3153

> <EXACT_MASS>
245.162708229

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
40

> <JCHEM_AVERAGE_POLARIZABILITY>
26.514293565015066

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
0

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

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

> <ALOGPS_LOGP>
-2.00

> <JCHEM_LOGP>
-2.758289847471746

> <ALOGPS_LOGS>
-4.07

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.342854307487348

> <JCHEM_PKA_STRONGEST_BASIC>
-7.092942553084254

> <JCHEM_POLAR_SURFACE_AREA>
66.43

> <JCHEM_REFRACTIVITY>
86.4392

> <JCHEM_ROTATABLE_BOND_COUNT>
8

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
2.54e-02 g/l

> <JCHEM_TRADITIONAL_IUPAC>
2-methylbutyroylcarnitine

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000378
     RDKit          3D
2-Methylbutyroylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    3.8272    2.3739   -0.0034 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6710    1.4507   -0.3578 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.7721    0.2427    0.5122 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1237   -0.4077    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6665   -0.7293    0.3328 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8822   -1.9273   -0.0371 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.3415   -0.4549    0.5380 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7193   -1.3863    0.3675 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4271   -1.7139    1.6204 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5203   -2.6932    1.4567 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8729   -3.2749    0.4249 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2788   -3.0303    2.6033 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.5506   -0.9783   -0.8085 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1799    0.2800   -0.7574 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.3733    1.3459   -0.2161 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4269    0.6809   -2.1587 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5022    0.2508   -0.1340 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5485    3.4299   -0.2057 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7214    2.1636   -0.6384 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0502    2.3182    1.0816 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7375    2.0190   -0.0800 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6395    1.2424   -1.4253 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7750    0.5457    1.5727 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4088   -0.2810   -0.8509 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9137    0.0193    0.8467 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0860   -1.5064    0.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2067   -2.3151    0.0484 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6761   -2.2258    2.2948 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8640   -0.8440    2.1512 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2423   -1.7522   -1.1267 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7973   -0.9177   -1.6617 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4422    1.3934   -0.8424 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1843    1.2836    0.8521 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8602    2.3465   -0.3909 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1504    1.5099   -2.1849 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4376    1.0054   -2.5427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8321   -0.1438   -2.7520 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9443   -0.7705   -0.3283 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4731    0.4934    0.9351 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2028    0.9572   -0.6202 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  2 22  1  0
  3 23  1  0
  4 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

HEADER    PROTEIN                                 26-MAY-19   NONE
TITLE     NULL                                                        
COMPND    MOLECULE: HMDB00378.mol                                     
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   26-MAY-19         0                                  
HETATM    1  N   UNK     0      -2.001   0.770   0.000  0.00  0.00           N+1
HETATM    2  C   UNK     0      -0.667   0.000   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0       0.667   0.770   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0       0.667   2.310   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0       2.001   0.000   0.000  0.00  0.00           O+0
HETATM    6  C   UNK     0       2.001  -1.540   0.000  0.00  0.00           C+0
HETATM    7  C   UNK     0       0.667  -2.310   0.000  0.00  0.00           C+0
HETATM    8  C   UNK     0       0.667  -3.850   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0      -0.667  -4.620   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0      -0.667  -1.540   0.000  0.00  0.00           C+0
HETATM   11  O   UNK     0       3.334  -2.310   0.000  0.00  0.00           O+0
HETATM   12  C   UNK     0      -3.334   0.000   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0      -2.990   1.950   0.000  0.00  0.00           C+0
HETATM   14  C   UNK     0      -1.011   1.950   0.000  0.00  0.00           C+0
HETATM   15  C   UNK     0       2.001   3.080   0.000  0.00  0.00           C+0
HETATM   16  O   UNK     0       3.334   2.310   0.000  0.00  0.00           O-1
HETATM   17  O   UNK     0       2.001   4.620   0.000  0.00  0.00           O+0
CONECT    1    2   12   13   14                                             
CONECT    2    1    3                                                       
CONECT    3    2    4    5                                                  
CONECT    4    3   15                                                       
CONECT    5    3    6                                                       
CONECT    6    5    7   11                                                  
CONECT    7    6    8   10                                                  
CONECT    8    7    9                                                       
CONECT    9    8                                                            
CONECT   10    7                                                            
CONECT   11    6                                                            
CONECT   12    1                                                            
CONECT   13    1                                                            
CONECT   14    1                                                            
CONECT   15    4   16   17                                                  
CONECT   16   15                                                            
CONECT   17   15                                                            
MASTER        0    0    0    0    0    0    0    0   17    0   32    0
END

COMPND    HMDB0000378
HETATM    1  C1  UNL     1       3.827   2.374  -0.003  1.00  0.00           C  
HETATM    2  C2  UNL     1       2.671   1.451  -0.358  1.00  0.00           C  
HETATM    3  C3  UNL     1       2.772   0.243   0.512  1.00  0.00           C  
HETATM    4  C4  UNL     1       4.124  -0.408   0.215  1.00  0.00           C  
HETATM    5  C5  UNL     1       1.667  -0.729   0.333  1.00  0.00           C  
HETATM    6  O1  UNL     1       1.882  -1.927  -0.037  1.00  0.00           O  
HETATM    7  O2  UNL     1       0.342  -0.455   0.538  1.00  0.00           O  
HETATM    8  C6  UNL     1      -0.719  -1.386   0.368  1.00  0.00           C  
HETATM    9  C7  UNL     1      -1.427  -1.714   1.620  1.00  0.00           C  
HETATM   10  C8  UNL     1      -2.520  -2.693   1.457  1.00  0.00           C  
HETATM   11  O3  UNL     1      -2.873  -3.275   0.425  1.00  0.00           O  
HETATM   12  O4  UNL     1      -3.279  -3.030   2.603  1.00  0.00           O1-
HETATM   13  C9  UNL     1      -1.551  -0.978  -0.808  1.00  0.00           C  
HETATM   14  N1  UNL     1      -2.180   0.280  -0.757  1.00  0.00           N1+
HETATM   15  C10 UNL     1      -1.373   1.346  -0.216  1.00  0.00           C  
HETATM   16  C11 UNL     1      -2.427   0.681  -2.159  1.00  0.00           C  
HETATM   17  C12 UNL     1      -3.502   0.251  -0.134  1.00  0.00           C  
HETATM   18  H1  UNL     1       3.548   3.430  -0.206  1.00  0.00           H  
HETATM   19  H2  UNL     1       4.721   2.164  -0.638  1.00  0.00           H  
HETATM   20  H3  UNL     1       4.050   2.318   1.082  1.00  0.00           H  
HETATM   21  H4  UNL     1       1.738   2.019  -0.080  1.00  0.00           H  
HETATM   22  H5  UNL     1       2.639   1.242  -1.425  1.00  0.00           H  
HETATM   23  H6  UNL     1       2.775   0.546   1.573  1.00  0.00           H  
HETATM   24  H7  UNL     1       4.409  -0.281  -0.851  1.00  0.00           H  
HETATM   25  H8  UNL     1       4.914   0.019   0.847  1.00  0.00           H  
HETATM   26  H9  UNL     1       4.086  -1.506   0.418  1.00  0.00           H  
HETATM   27  H10 UNL     1      -0.207  -2.315   0.048  1.00  0.00           H  
HETATM   28  H11 UNL     1      -0.676  -2.226   2.295  1.00  0.00           H  
HETATM   29  H12 UNL     1      -1.864  -0.844   2.151  1.00  0.00           H  
HETATM   30  H13 UNL     1      -2.242  -1.752  -1.127  1.00  0.00           H  
HETATM   31  H14 UNL     1      -0.797  -0.918  -1.662  1.00  0.00           H  
HETATM   32  H15 UNL     1      -0.442   1.393  -0.842  1.00  0.00           H  
HETATM   33  H16 UNL     1      -1.184   1.284   0.852  1.00  0.00           H  
HETATM   34  H17 UNL     1      -1.860   2.347  -0.391  1.00  0.00           H  
HETATM   35  H18 UNL     1      -3.150   1.510  -2.185  1.00  0.00           H  
HETATM   36  H19 UNL     1      -1.438   1.005  -2.543  1.00  0.00           H  
HETATM   37  H20 UNL     1      -2.832  -0.144  -2.752  1.00  0.00           H  
HETATM   38  H21 UNL     1      -3.944  -0.770  -0.328  1.00  0.00           H  
HETATM   39  H22 UNL     1      -3.473   0.493   0.935  1.00  0.00           H  
HETATM   40  H23 UNL     1      -4.203   0.957  -0.620  1.00  0.00           H  
CONECT    1    2   18   19   20
CONECT    2    3   21   22
CONECT    3    4    5   23
CONECT    4   24   25   26
CONECT    5    6    6    7
CONECT    7    8
CONECT    8    9   13   27
CONECT    9   10   28   29
CONECT   10   11   11   12
CONECT   13   14   30   31
CONECT   14   15   16   17
CONECT   15   32   33   34
CONECT   16   35   36   37
CONECT   17   38   39   40
END

HMDB0000378
     RDKit          3D
2-Methylbutyroylcarnitine
 40 39  0  0  0  0  0  0  0  0999 V2000
    3.8272    2.3739   -0.0034 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.6710    1.4507   -0.3578 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.7721    0.2427    0.5122 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.1237   -0.4077    0.2154 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6665   -0.7293    0.3328 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8822   -1.9273   -0.0371 O   0  0  0  0  0  0  0  0  0  0  0  0
    0.3415   -0.4549    0.5380 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7193   -1.3863    0.3675 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4271   -1.7139    1.6204 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5203   -2.6932    1.4567 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8729   -3.2749    0.4249 O   0  0  0  0  0  0  0  0  0  0  0  0
   -3.2788   -3.0303    2.6033 O   0  0  0  0  0  1  0  0  0  0  0  0
   -1.5506   -0.9783   -0.8085 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1799    0.2800   -0.7574 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.3733    1.3459   -0.2161 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4269    0.6809   -2.1587 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5022    0.2508   -0.1340 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5485    3.4299   -0.2057 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7214    2.1636   -0.6384 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0502    2.3182    1.0816 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.7375    2.0190   -0.0800 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6395    1.2424   -1.4253 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7750    0.5457    1.5727 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.4088   -0.2810   -0.8509 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.9137    0.0193    0.8467 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.0860   -1.5064    0.4177 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2067   -2.3151    0.0484 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6761   -2.2258    2.2948 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8640   -0.8440    2.1512 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2423   -1.7522   -1.1267 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7973   -0.9177   -1.6617 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.4422    1.3934   -0.8424 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1843    1.2836    0.8521 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.8602    2.3465   -0.3909 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1504    1.5099   -2.1849 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.4376    1.0054   -2.5427 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8321   -0.1438   -2.7520 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.9443   -0.7705   -0.3283 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4731    0.4934    0.9351 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2028    0.9572   -0.6202 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  1  0
  3  5  1  0
  5  6  2  0
  5  7  1  0
  7  8  1  0
  8  9  1  0
  9 10  1  0
 10 11  2  0
 10 12  1  0
  8 13  1  0
 13 14  1  0
 14 15  1  0
 14 16  1  0
 14 17  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  0
  2 22  1  0
  3 23  1  0
  4 24  1  0
  4 25  1  0
  4 26  1  0
  8 27  1  0
  9 28  1  0
  9 29  1  0
 13 30  1  0
 13 31  1  0
 15 32  1  0
 15 33  1  0
 15 34  1  0
 16 35  1  0
 16 36  1  0
 16 37  1  0
 17 38  1  0
 17 39  1  0
 17 40  1  0
M  CHG  2  12  -1  14   1
M  END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoate	ChEBI
3-[(2-Methylbutanoyl)oxy]-4-(trimethylammonio)butanoic acid	Generator
(2-Methylbutyryl)carnitine	HMDB
L-2-Methylbutyrate (3-carboxy-2-hydroxypropyl)trimethyl-hydroxide ammonium inner salt	HMDB
Methylbutyroyl-carnitine	HMDB

Chemical Formula

C₁₂H₂₃NO₄

Average Molecular Weight

245.3153

Monoisotopic Molecular Weight

245.162708229

IUPAC Name

3-[(2-methylbutanoyl)oxy]-4-(trimethylazaniumyl)butanoate

Traditional Name

2-methylbutyroylcarnitine

CAS Registry Number

31023-25-3

SMILES

CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C

InChI Identifier

InChI=1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3

InChI Key

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

C5-acylcarnitine (CHEBI:73026 )

Ontology

Physiological effect

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 32966057)

Cellular substructure

Organelle

Peroxisome (PMID: 35710135)

Organ

Placenta (HMDB: HMDB0000378)

Excreta

Biofluid or Excreta

Urine (PMID: 21389975)

Cell

All cells and tissues (PMID: 35710135)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000378)

Source

Endogenous

Endogenous (HMDB: HMDB0000378)

Animal

Animal (HMDB: HMDB0000378)

Exogenous

Food

Food (HMDB: HMDB0000378)

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: HMDB0000378)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0000378)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0000378)

Lipid metabolism pathway (HMDB: HMDB0000378)

Cellular process

Cell signaling (HMDB: HMDB0000378)

Biochemical process

Lipid transport (HMDB: HMDB0000378)

Role

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	0.025 g/L	ALOGPS
logP	-2	ALOGPS
logP	-2.8	ChemAxon
logS	-4.1	ALOGPS
pKa (Strongest Acidic)	4.34	ChemAxon
pKa (Strongest Basic)	-7.1	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	3	ChemAxon
Hydrogen Donor Count	0	ChemAxon
Polar Surface Area	66.43 Å²	ChemAxon
Rotatable Bond Count	8	ChemAxon
Refractivity	86.44 m³·mol⁻¹	ChemAxon
Polarizability	26.51 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
AllCCS	[M+H]+	158.736	32859911
AllCCS	[M-H]-	164.668	32859911
DeepCCS	[M+H]+	155.615	30932474
DeepCCS	[M-H]-	153.256	30932474
DeepCCS	[M-2H]-	186.14	30932474
DeepCCS	[M+Na]+	161.708	30932474
AllCCS	[M+H]+	158.7	32859911
AllCCS	[M+H-H2O]+	155.7	32859911
AllCCS	[M+NH4]+	161.5	32859911
AllCCS	[M+Na]+	162.4	32859911
AllCCS	[M-H]-	164.7	32859911
AllCCS	[M+Na-2H]-	165.7	32859911
AllCCS	[M+HCOO]-	167.0	32859911

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.	2.42 minutes	32390414
Predicted by Siyang on May 30, 2022	10.7097 minutes	33406817
Predicted by Siyang using ReTip algorithm on June 8, 2022	4.6 minutes	32390414
AjsUoB = Accucore 150 Amide HILIC with 10mM Ammonium Formate, 0.1% Formic Acid	216.5 seconds	40023050
Fem_Long = Waters ACQUITY UPLC HSS T3 C18 with Water:MeOH and 0.1% Formic Acid	1140.4 seconds	40023050
Fem_Lipids = Ascentis Express C18 with (60:40 water:ACN):(90:10 IPA:ACN) and 10mM NH4COOH + 0.1% Formic Acid	198.9 seconds	40023050
Life_Old = Waters ACQUITY UPLC BEH C18 with Water:(20:80 acetone:ACN) and 0.1% Formic Acid	129.9 seconds	40023050
Life_New = RP Waters ACQUITY UPLC HSS T3 C18 with Water:(30:70 MeOH:ACN) and 0.1% Formic Acid	154.0 seconds	40023050
RIKEN = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	71.3 seconds	40023050
Eawag_XBridgeC18 = XBridge C18 3.5u 2.1x50 mm with Water:MeOH and 0.1% Formic Acid	402.1 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	398.8 seconds	40023050
HILIC_BDD_2 = Merck SeQuant ZIC-HILIC with ACN(0.1% formic acid):water(16 mM ammonium formate)	567.0 seconds	40023050
UniToyama_Atlantis = RP Waters Atlantis T3 (2.1 x 150 mm, 5 um) with ACN:Water and 0.1% Formic Acid	959.9 seconds	40023050
BDD_C18 = Hypersil Gold 1.9µm C18 with Water:ACN and 0.1% Formic Acid	289.3 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	1484.4 seconds	40023050
SNU_RIKEN_POS = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	204.7 seconds	40023050
RPMMFDA = Waters ACQUITY UPLC BEH C18 with Water:ACN and 0.1% Formic Acid	268.2 seconds	40023050
MTBLS87 = Merck SeQuant ZIC-pHILIC column with ACN:Water and :ammonium carbonate	320.4 seconds	40023050
KI_GIAR_zic_HILIC_pH2_7 = Merck SeQuant ZIC-HILIC with ACN:Water and 0.1% FA	227.4 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	26.0 seconds	40023050

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
2-Methylbutyroylcarnitine	CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C	1893.3	Standard polar	33892256
2-Methylbutyroylcarnitine	CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C	1312.7	Standard non polar	33892256
2-Methylbutyroylcarnitine	CCC(C)C(=O)OC(CC([O-])=O)C[N+](C)(C)C	1513.3	Semi standard non polar	33892256

Spectra

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 but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	21389975	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Urine	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Autosomal dominant polycystic kidney disease (ADPKD)	21389975	details

Associated Disorders and Diseases

Disease References

Autosomal dominant polycystic kidney disease
Gronwald W, Klein MS, Zeltner R, Schulze BD, Reinhold SW, Deutschmann M, Immervoll AK, Boger CA, Banas B, Eckardt KU, Oefner PJ: Detection of autosomal dominant polycystic kidney disease by NMR spectroscopic fingerprinting of urine. Kidney Int. 2011 Jun;79(11):1244-53. doi: 10.1038/ki.2011.30. Epub 2011 Mar 9. [PubMed:21389975 ]

Associated OMIM IDs

601313 (Autosomal dominant polycystic kidney disease)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021996

KNApSAcK ID

Not Available

Chemspider ID

4932320

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5367

PubChem Compound

6426901

PDB ID

Not Available

ChEBI ID

73026

Food Biomarker Ontology

Not Available

VMH ID

Not Available

MarkerDB ID

Not Available

Good Scents ID

Not Available

References

Synthesis Reference

Nakanishi, Toyofumi; Shimizu, Akira; Arimoto, Masao; Kanai, Michiko. Synthesis of acylcarnitines for differential diagnosis of metabolic disorders. Nippon Iyo Masu Supekutoru Gakkai Koenshu (1993), 18 129-32.

Material Safety Data Sheet (MSDS)

Not Available

General References

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 ]
Salamino F, Di Lisa F, Burlina AB, Menabo R, Barbato R, De Tullio R, Siliprandi N: Involvement of erythrocyte calpain in glycine- and carnitine-treated isovaleric acidemia. Pediatr Res. 1994 Aug;36(2):182-6. [PubMed:7970932 ]
Abdenur JE, Chamoles NA, Guinle AE, Schenone AB, Fuertes AN: Diagnosis of isovaleric acidaemia by tandem mass spectrometry: false positive result due to pivaloylcarnitine in a newborn screening programme. J Inherit Metab Dis. 1998 Aug;21(6):624-30. [PubMed:9762597 ]
Fries MH, Rinaldo P, Schmidt-Sommerfeld E, Jurecki E, Packman S: Isovaleric acidemia: response to a leucine load after three weeks of supplementation with glycine, L-carnitine, and combined glycine-carnitine therapy. J Pediatr. 1996 Sep;129(3):449-52. [PubMed:8804338 ]
Millington DS, Roe CR, Maltby DA, Inoue F: Endogenous catabolism is the major source of toxic metabolites in isovaleric acidemia. J Pediatr. 1987 Jan;110(1):56-60. [PubMed:3794887 ]
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Showing metabocard for 2-Methylbutyroylcarnitine (HMDB0000378)

MOL for HMDB0000378 (2-Methylbutyroylcarnitine)

3D MOL for HMDB0000378 (2-Methylbutyroylcarnitine)

3D SDF for HMDB0000378 (2-Methylbutyroylcarnitine)

3D-SDF for HMDB0000378 (2-Methylbutyroylcarnitine)

PDB for HMDB0000378 (2-Methylbutyroylcarnitine)

3D PDB for HMDB0000378 (2-Methylbutyroylcarnitine)

SMILES for HMDB0000378 (2-Methylbutyroylcarnitine)

INCHI for HMDB0000378 (2-Methylbutyroylcarnitine)

Structure for HMDB0000378 (2-Methylbutyroylcarnitine)

3D Structure for HMDB0000378 (2-Methylbutyroylcarnitine)

Predicted Collision Cross Sections

Predicted Retention Times

Underivatized

Predicted Kovats Retention Indices

Underivatized