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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

HMDB0000824

Secondary Accession Numbers

HMDB0062514
HMDB00824
HMDB62514

Metabolite Identification

Common Name

Propionylcarnitine

Description

Propionylcarnitine is an acylcarnitine. More specifically, it is an propanoic 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. Propionylcarnitine is therefore classified as a short chain AC. As a short-chain acylcarnitine propionylcarnitine 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 propionylcarnitine is elevated in the blood or plasma of individuals with methylmalonic academia/methylmalonyl-CoA mutase deficiency (PMID: 26454439 , PMID: 25750861 , PMID: 31622506 , PMID: 30712249 ), propionic academia/mitochondrial propionyl-CoA carboxylase deficiency (PMID: 31715057 , PMID: 28915261 ), obesity and Type 2 Diabetes Mellitus (PMID: 30186675 ), cobalamin C deficiency (PMID: 25367534 ), chronic heart failure (PMID: 22622056 ), diastolic heart failure (PMID: 26010610 ), and systolic heart failure (PMID: 26010610 ). It is also decreased in the blood or plasma of individuals with familial Mediterranean fever (PMID: 29900937 ) and type 2 diabetes Mellitus (PMID: 19369366 ). It is also decreased in the urine of individuals with obesity (PMID: 26910390 ). Propionylcarnitine is also found to be associated with propionic acidemia, which is an inborn error of metabolism. 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

HMDB0000824
     RDKit          3D
Propionylcarnitine
 35 34  0  0  0  0  0  0  0  0999 V2000
    4.4053    0.3898   -0.9844 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5343   -0.8411   -1.0792 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1165   -0.3707   -1.2688 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6539   -0.0188   -2.3634 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3067   -0.3352   -0.1689 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0694    0.0982   -0.1908 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.0260    1.3613    0.7008 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8951    2.3407    0.0786 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7687    2.6294   -1.1387 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.9239    2.9790    0.7802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8668   -0.9846    0.4176 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2904   -0.7895    0.4880 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.9567   -2.1014    0.5127 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6591   -0.1329    1.7307 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8442   -0.0451   -0.6050 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.3008    0.2973   -1.6246 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8465    1.3019   -1.3183 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7659    0.5961    0.0475 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6246   -1.4813   -0.1709 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8305   -1.4797   -1.9344 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2785    0.3659   -1.2241 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0561    1.7684    0.8060 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3194    1.1036    1.7230 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7741    3.2429    0.2760 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5027   -1.1226    1.4723 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6976   -1.9657   -0.0646 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1736   -2.8753    0.4477 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6726   -2.2178   -0.3121 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5109   -2.2712    1.4609 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1807    0.8235    1.5191 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3768   -0.7556    2.3184 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7352    0.0382    2.3159 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8180   -0.4931   -0.8808 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1139    0.9879   -0.2491 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2372   -0.0427   -1.5172 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  2  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
  6 11  1  0
 11 12  1  0
 12 13  1  0
 12 14  1  0
 12 15  1  0
  1 16  1  0
  1 17  1  0
  1 18  1  0
  2 19  1  0
  2 20  1  0
  6 21  1  6
  7 22  1  0
  7 23  1  0
 10 24  1  0
 11 25  1  0
 11 26  1  0
 13 27  1  0
 13 28  1  0
 13 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
M  CHG  1  12   1
M  END

  Mrv1652303062020122D          

 15 14  0  0  0  0            999 V2000
 9996.603610001.0029    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
 9997.317710000.5896    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9998.031810001.0029    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.745910000.5896    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9999.459910001.0029    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9998.031810001.8274    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 9996.603610001.8274    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.889510002.2379    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
 9995.171310001.8253    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.889510003.0629    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.036410002.7309    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.897810000.5793    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9995.8978 9999.7549    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 9996.6119 9999.3415    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
 9995.1837 9999.3415    0.0000 O   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 14  1  0  0  0  0
 13 15  2  0  0  0  0
M  CHG  2   8   1  14  -1
M  END
> <DATABASE_ID>
HMDB0000824

> <DATABASE_NAME>
hmdb

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

> <INCHI_IDENTIFIER>
InChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3/t8-/m1/s1

> <INCHI_KEY>
UFAHZIUFPNSHSL-MRVPVSSYSA-N

> <FORMULA>
C10H19NO4

> <MOLECULAR_WEIGHT>
217.265

> <EXACT_MASS>
217.131408096

> <JCHEM_ACCEPTOR_COUNT>
3

> <JCHEM_ATOM_COUNT>
34

> <JCHEM_AVERAGE_POLARIZABILITY>
22.682257365808457

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
0

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
(3R)-3-(propanoyloxy)-4-(trimethylazaniumyl)butanoate

> <ALOGPS_LOGP>
-2.27

> <JCHEM_LOGP>
-3.7458447908050787

> <ALOGPS_LOGS>
-3.25

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA_STRONGEST_ACIDIC>
4.185139998188834

> <JCHEM_PKA_STRONGEST_BASIC>
-7.054133284063387

> <JCHEM_POLAR_SURFACE_AREA>
66.43

> <JCHEM_REFRACTIVITY>
77.2637

> <JCHEM_ROTATABLE_BOND_COUNT>
7

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.52e-01 g/l

> <JCHEM_TRADITIONAL_IUPAC>
O-propanoylcarnitine

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000824
     RDKit          3D
Propionylcarnitine
 35 34  0  0  0  0  0  0  0  0999 V2000
    4.4053    0.3898   -0.9844 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5343   -0.8411   -1.0792 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1165   -0.3707   -1.2688 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6539   -0.0188   -2.3634 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3067   -0.3352   -0.1689 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0694    0.0982   -0.1908 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.0260    1.3613    0.7008 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8951    2.3407    0.0786 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7687    2.6294   -1.1387 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.9239    2.9790    0.7802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8668   -0.9846    0.4176 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2904   -0.7895    0.4880 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.9567   -2.1014    0.5127 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6591   -0.1329    1.7307 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8442   -0.0451   -0.6050 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.3008    0.2973   -1.6246 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8465    1.3019   -1.3183 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7659    0.5961    0.0475 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6246   -1.4813   -0.1709 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8305   -1.4797   -1.9344 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2785    0.3659   -1.2241 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0561    1.7684    0.8060 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3194    1.1036    1.7230 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7741    3.2429    0.2760 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5027   -1.1226    1.4723 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6976   -1.9657   -0.0646 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1736   -2.8753    0.4477 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6726   -2.2178   -0.3121 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5109   -2.2712    1.4609 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1807    0.8235    1.5191 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3768   -0.7556    2.3184 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7352    0.0382    2.3159 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8180   -0.4931   -0.8808 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1139    0.9879   -0.2491 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2372   -0.0427   -1.5172 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  2  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
  6 11  1  0
 11 12  1  0
 12 13  1  0
 12 14  1  0
 12 15  1  0
  1 16  1  0
  1 17  1  0
  1 18  1  0
  2 19  1  0
  2 20  1  0
  6 21  1  6
  7 22  1  0
  7 23  1  0
 10 24  1  0
 11 25  1  0
 11 26  1  0
 13 27  1  0
 13 28  1  0
 13 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
M  CHG  1  12   1
M  END

HEADER    PROTEIN                                 06-MAR-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   06-MAR-20         0                                  
HETATM    1  C   UNK     0    8660.3278668.539   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0    8661.6608667.767   0.000  0.00  0.00           O+0
HETATM    3  C   UNK     0    8662.9938668.539   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8664.3268667.767   0.000  0.00  0.00           C+0
HETATM    5  C   UNK     0    8665.6588668.539   0.000  0.00  0.00           C+0
HETATM    6  O   UNK     0    8662.9938670.078   0.000  0.00  0.00           O+0
HETATM    7  C   UNK     0    8660.3278670.078   0.000  0.00  0.00           C+0
HETATM    8  N   UNK     0    8658.9948670.844   0.000  0.00  0.00           N+1
HETATM    9  C   UNK     0    8657.6538670.074   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0    8658.9948672.384   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0    8657.4018671.764   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0    8659.0098667.748   0.000  0.00  0.00           C+0
HETATM   13  C   UNK     0    8659.0098666.209   0.000  0.00  0.00           C+0
HETATM   14  O   UNK     0    8660.3428665.437   0.000  0.00  0.00           O-1
HETATM   15  O   UNK     0    8657.6768665.437   0.000  0.00  0.00           O+0
CONECT    1    2    7   12                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    6                                                  
CONECT    4    3    5                                                       
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   14   15                                                  
CONECT   14   13                                                            
CONECT   15   13                                                            
MASTER        0    0    0    0    0    0    0    0   15    0   28    0
END

COMPND    HMDB0000824
HETATM    1  C1  UNL     1       4.405   0.390  -0.984  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.534  -0.841  -1.079  1.00  0.00           C  
HETATM    3  C3  UNL     1       2.117  -0.371  -1.269  1.00  0.00           C  
HETATM    4  O1  UNL     1       1.654  -0.019  -2.363  1.00  0.00           O  
HETATM    5  O2  UNL     1       1.307  -0.335  -0.169  1.00  0.00           O  
HETATM    6  C4  UNL     1      -0.069   0.098  -0.191  1.00  0.00           C  
HETATM    7  C5  UNL     1      -0.026   1.361   0.701  1.00  0.00           C  
HETATM    8  C6  UNL     1       0.895   2.341   0.079  1.00  0.00           C  
HETATM    9  O3  UNL     1       0.769   2.629  -1.139  1.00  0.00           O  
HETATM   10  O4  UNL     1       1.924   2.979   0.780  1.00  0.00           O  
HETATM   11  C7  UNL     1      -0.867  -0.985   0.418  1.00  0.00           C  
HETATM   12  N1  UNL     1      -2.290  -0.790   0.488  1.00  0.00           N1+
HETATM   13  C8  UNL     1      -2.957  -2.101   0.513  1.00  0.00           C  
HETATM   14  C9  UNL     1      -2.659  -0.133   1.731  1.00  0.00           C  
HETATM   15  C10 UNL     1      -2.844  -0.045  -0.605  1.00  0.00           C  
HETATM   16  H1  UNL     1       5.301   0.297  -1.625  1.00  0.00           H  
HETATM   17  H2  UNL     1       3.847   1.302  -1.318  1.00  0.00           H  
HETATM   18  H3  UNL     1       4.766   0.596   0.048  1.00  0.00           H  
HETATM   19  H4  UNL     1       3.625  -1.481  -0.171  1.00  0.00           H  
HETATM   20  H5  UNL     1       3.831  -1.480  -1.934  1.00  0.00           H  
HETATM   21  H6  UNL     1      -0.279   0.366  -1.224  1.00  0.00           H  
HETATM   22  H7  UNL     1      -1.056   1.768   0.806  1.00  0.00           H  
HETATM   23  H8  UNL     1       0.319   1.104   1.723  1.00  0.00           H  
HETATM   24  H9  UNL     1       2.774   3.243   0.276  1.00  0.00           H  
HETATM   25  H10 UNL     1      -0.503  -1.123   1.472  1.00  0.00           H  
HETATM   26  H11 UNL     1      -0.698  -1.966  -0.065  1.00  0.00           H  
HETATM   27  H12 UNL     1      -2.174  -2.875   0.448  1.00  0.00           H  
HETATM   28  H13 UNL     1      -3.673  -2.218  -0.312  1.00  0.00           H  
HETATM   29  H14 UNL     1      -3.511  -2.271   1.461  1.00  0.00           H  
HETATM   30  H15 UNL     1      -3.181   0.823   1.519  1.00  0.00           H  
HETATM   31  H16 UNL     1      -3.377  -0.756   2.318  1.00  0.00           H  
HETATM   32  H17 UNL     1      -1.735   0.038   2.316  1.00  0.00           H  
HETATM   33  H18 UNL     1      -3.818  -0.493  -0.881  1.00  0.00           H  
HETATM   34  H19 UNL     1      -3.114   0.988  -0.249  1.00  0.00           H  
HETATM   35  H20 UNL     1      -2.237  -0.043  -1.517  1.00  0.00           H  
CONECT    1    2   16   17   18
CONECT    2    3   19   20
CONECT    3    4    4    5
CONECT    5    6
CONECT    6    7   11   21
CONECT    7    8   22   23
CONECT    8    9    9   10
CONECT   10   24
CONECT   11   12   25   26
CONECT   12   13   14   15
CONECT   13   27   28   29
CONECT   14   30   31   32
CONECT   15   33   34   35
END

HMDB0000824
     RDKit          3D
Propionylcarnitine
 35 34  0  0  0  0  0  0  0  0999 V2000
    4.4053    0.3898   -0.9844 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.5343   -0.8411   -1.0792 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.1165   -0.3707   -1.2688 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.6539   -0.0188   -2.3634 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.3067   -0.3352   -0.1689 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0694    0.0982   -0.1908 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.0260    1.3613    0.7008 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.8951    2.3407    0.0786 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.7687    2.6294   -1.1387 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.9239    2.9790    0.7802 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8668   -0.9846    0.4176 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2904   -0.7895    0.4880 N   0  0  0  0  0  4  0  0  0  0  0  0
   -2.9567   -2.1014    0.5127 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6591   -0.1329    1.7307 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.8442   -0.0451   -0.6050 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.3008    0.2973   -1.6246 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8465    1.3019   -1.3183 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.7659    0.5961    0.0475 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.6246   -1.4813   -0.1709 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8305   -1.4797   -1.9344 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2785    0.3659   -1.2241 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.0561    1.7684    0.8060 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.3194    1.1036    1.7230 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.7741    3.2429    0.2760 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.5027   -1.1226    1.4723 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.6976   -1.9657   -0.0646 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1736   -2.8753    0.4477 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.6726   -2.2178   -0.3121 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5109   -2.2712    1.4609 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1807    0.8235    1.5191 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.3768   -0.7556    2.3184 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7352    0.0382    2.3159 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8180   -0.4931   -0.8808 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1139    0.9879   -0.2491 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2372   -0.0427   -1.5172 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  3  4  2  0
  3  5  1  0
  5  6  1  0
  6  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
  6 11  1  0
 11 12  1  0
 12 13  1  0
 12 14  1  0
 12 15  1  0
  1 16  1  0
  1 17  1  0
  1 18  1  0
  2 19  1  0
  2 20  1  0
  6 21  1  6
  7 22  1  0
  7 23  1  0
 10 24  1  0
 11 25  1  0
 11 26  1  0
 13 27  1  0
 13 28  1  0
 13 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
M  CHG  1  12   1
M  END

View in JSmol View NMR Assignments View Stereo Labels

Synonyms

Value	Source
(3R)-3-(Propionyloxy)-4-(trimethylammonio)butanoate	ChEBI
(R)-3-Carboxy-N,N,N-trimethyl-2-(1-oxopropoxy)-1-propanaminium inner salt	ChEBI
O-Propanoyl-(R)-carnitine	ChEBI
O-Propanoyl-carnitine	ChEBI
O-Propanoylcarnitine	ChEBI
O-Propinoyl-L-carnitine	ChEBI
(3R)-3-(Propionyloxy)-4-(trimethylammonio)butanoic acid	Generator
(-)-Propionylcarnitine	HMDB
C3-Carnitine	HMDB
L-Carnitine propionyl ester	HMDB
L-Propionylcarnitine	HMDB
Levocarnitine propil	HMDB
O-Propanoyl-L-carnitine	HMDB
O-Propionyl-L-carnitine	HMDB
Propanoyl-L-carnitine	HMDB
Propionyl-L-carnitine	HMDB
Propionylcarnitine	HMDB

Chemical Formula

C₁₀H₁₉NO₄

Average Molecular Weight

217.265

Monoisotopic Molecular Weight

217.131408096

IUPAC Name

(3R)-3-(propanoyloxy)-4-(trimethylazaniumyl)butanoate

Traditional Name

O-propanoylcarnitine

CAS Registry Number

20064-19-1

SMILES

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

InChI Identifier

InChI=1S/C10H19NO4/c1-5-10(14)15-8(6-9(12)13)7-11(2,3)4/h8H,5-7H2,1-4H3/t8-/m1/s1

InChI Key

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

O-acyl-L-carnitine (CHEBI:53210 )
O-propanoylcarnitine (CHEBI:53210 )
Fatty acyl carnitines (LMFA07070005 )

Ontology

Physiological effect

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 16425363)
Cerebrospinal Fluid (CSF) (PMID: 22546835)
Saliva (PMID: 22727327)
Cerebrospinal fluid (HMDB: HMDB0000824)

Cellular substructure

Organelle

Organ

Placenta (HMDB: HMDB0000824)

Excreta

Biofluid or Excreta

Urine (PMID: 24023812)
Feces (PMID: 27609529)

Cell

All cells and tissues (PMID: 35710135)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000824)

Source

Exogenous

Exogenous (HMDB: HMDB0000824)

Food

Food (HMDB: HMDB0000824)

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

Endogenous

Endogenous (HMDB: HMDB0000824)

Animal

Animal (HMDB: HMDB0000824)

Process

Role

Industrial application

Manufacturing industry

Material processing agent

Coloring agent

Fluorescent agent

Analgesic (HMDB: HMDB0000824)
Analgesic (HMDB: HMDB0000824)

Fluid processing agent

Surfactant

Surfactant (HMDB: HMDB0000824)

Pharmaceutical industry

Peripheral nervous system drug (HMDB: HMDB0000824)
Cardiotonic drug (HMDB: HMDB0000824)
Antirheumatic drug (HMDB: HMDB0000824)

Biomarker

Breast cancer ()
Diabetes mellitus type 2 ()
Cardiovascular disease ()
Renal cell carcinoma ()
Heart failure ()
Celiac disease ()
Inflammatory bowel disease ()
Obesity (MarkerDB: MDB00000262)
Propionic acidemia (MarkerDB: MDB00000262)

Biological role

Energy source (HMDB: HMDB0000824)
Energy source (PMID: 35710135)

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	0.15 g/L	ALOGPS
logP	-2.3	ALOGPS
logP	-3.7	ChemAxon
logS	-3.2	ALOGPS
pKa (Strongest Acidic)	4.19	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	77.26 m³·mol⁻¹	ChemAxon
Polarizability	22.68 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]+	149.047	30932474
DeepCCS	[M-H]-	146.711	30932474
DeepCCS	[M-2H]-	179.948	30932474
DeepCCS	[M+Na]+	154.959	30932474

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
Propionylcarnitine	CCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	2423.5	Standard polar	33892256
Propionylcarnitine	CCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	1335.5	Standard non polar	33892256
Propionylcarnitine	CCC(=O)O[C@H](CC(O)=O)C[N+](C)(C)C	1538.9	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Propionylcarnitine,1TMS,isomer #1	CCC(=O)O[C@H](CC(=O)O[Si](C)(C)C)C[N+](C)(C)C	1528.8	Semi standard non polar	33892256
Propionylcarnitine,1TBDMS,isomer #1	CCC(=O)O[C@H](CC(=O)O[Si](C)(C)C(C)(C)C)C[N+](C)(C)C	1751.7	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 - Propionylcarnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Propionylcarnitine GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-06	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Propionylcarnitine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-06	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 - Propionylcarnitine 10V, Positive-QTOF	splash10-014i-0090000000-183878d9af472b731f0f	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Propionylcarnitine 20V, Positive-QTOF	splash10-00kr-9050000000-a5c35e074ce368a1496b	2021-09-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Propionylcarnitine 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)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane
Mitochondria
Peroxisome

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva
Urine

Tissue Locations

Placenta

Pathways

Name	SMPDB/PathBank	KEGG
Oxidation of Branched Chain Fatty Acids		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.31 +/- 0.15 uM	Adult (>18 years old)	Both	Normal	21359215	details
Blood	Detected and Quantified	0.400 +/- 0.021 uM	Adult (>18 years old)	Both	Normal	16425363	details
Blood	Detected and Quantified	0.15-0.70 uM	Adult (>18 years old)	Both	Normal	The Metabolo...	details
Blood	Detected and Quantified	0.24 (0.2-0.3) uM	Newborn (0-30 days old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	0.58 (0.47-0.75) uM	Infant (0-1 year old)	Not Available	Normal	25754623	details
Blood	Detected and Quantified	0.44(0.14) uM	Adult (>18 years old)	Both	Normal	26010610	details
Blood	Detected and Quantified	0.34 +/- 0.10 uM	Adult (>18 years old)	Both	Normal	28278231	details
Blood	Detected and Quantified	0.564 (0.209-1.159) uM	Children (1-13 years old)	Both	Normal	9034211	details
Blood	Detected and Quantified	0.15-0.70 uM	Adult (>18 years old)	Both	Normal	The Metabolo...	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.018 +/- 0.009 uM	Adult (>18 years old)	Not Specified	Normal	22546835	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Normal	27609529	details
Feces	Detected and Quantified	0.27 +/- 0.12 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	0.16 +/- 0.06 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Saliva	Detected and Quantified	0.068 +/- 0.054 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Normal	22308371	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Not Specified	Normal	22727327	details
Urine	Detected and Quantified	86.75 +/- 9.39 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Normal	26910390	details
Urine	Detected and Quantified	0.07 (0.01-0.20) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details
Urine	Detected and Quantified	0.01-0.07 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	0.03 +/- 0.02 umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	0.03 +/- 0.02 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
Urine	Detected and Quantified	0.4814 +/- 0.371 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Normal	Analysis of 30 no...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.247 +/- 0.014 uM	Adult (>18 years old)	Both	Celiac disease	16425363	details
Blood	Detected and Quantified	0.2075 (0.1802) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details
Blood	Detected and Quantified	0.58(0.2) uM	Adult (>18 years old)	Both	Heart failure with preserved ejection fraction	26010610	details
Blood	Detected and Quantified	0.63(0.32) uM	Adult (>18 years old)	Both	Heart failure with reduced ejection fraction	26010610	details
Blood	Detected and Quantified	0.331 +/- 0.124 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Blood	Detected and Quantified	0.384 +/- 0.122 uM	Children (1-13 years old)	Both	Obesity	Metabolomics reve...	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Crohns disease	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Ulcerative colitis	27609529	details
Feces	Detected but not Quantified	Not Quantified	Children (6 - 18 years old)	Not Specified	Unclassified IBD	27609529	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Attachment loss	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Missing teeth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Periodontal Probing Depth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Prosthesis/Missing teeth	31026179	details
Saliva	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Male	Tooth Decay	31026179	details
Urine	Detected and Quantified	142.23 +/- 13.71 umol/mmol creatinine	Adolescent (13-18 years old)	Both	Obese	26910390	details
Urine	Detected and Quantified	2.09 umol/mmol creatinine	Adult (>18 years old)	Not Specified	Propionic acidemia	28853722	details
Urine	Detected and Quantified	0.3638 +/- 0.6673 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Eosinophilic esophagitis	Analysis of 30 no...	details
Urine	Detected and Quantified	0.218 +/- 0.2137 umol/mmol creatinine	Children (1 - 13 years old)	Not Specified	Gastroesophageal reflux disease	Analysis of 30 no...	details

Associated Disorders and Diseases

Disease References

Celiac disease
Bene J, Komlosi K, Gasztonyi B, Juhasz M, Tulassay Z, Melegh B: Plasma carnitine ester profile in adult celiac disease patients maintained on long-term gluten free diet. World J Gastroenterol. 2005 Nov 14;11(42):6671-5. [PubMed:16425363 ]
Crohn's disease
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Ulcerative colitis
Kolho KL, Pessia A, Jaakkola T, de Vos WM, Velagapudi V: Faecal and Serum Metabolomics in Paediatric Inflammatory Bowel Disease. J Crohns Colitis. 2017 Mar 1;11(3):321-334. doi: 10.1093/ecco-jcc/jjw158. [PubMed:27609529 ]
Attachment loss
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Missing teeth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Periodontal Probing Depth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Prosthesis/Missing teeth
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Tooth Decay
Liebsch C, Pitchika V, Pink C, Samietz S, Kastenmuller G, Artati A, Suhre K, Adamski J, Nauck M, Volzke H, Friedrich N, Kocher T, Holtfreter B, Pietzner M: The Saliva Metabolome in Association to Oral Health Status. J Dent Res. 2019 Jun;98(6):642-651. doi: 10.1177/0022034519842853. Epub 2019 Apr 26. [PubMed:31026179 ]
Eosinophilic esophagitis
Slae, M., Huynh, H., Wishart, D.S. (2014). Analysis of 30 normal pediatric urine samples via NMR spectroscopy (unpublished work). NA.
Propionic acidemia
Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
Obesity
Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22. [PubMed:26910390 ] Simone Wahl, Christina Holzapfel, Zhonghao Yu, Michaela Breier, Ivan Kondofersky, Christiane Fuchs, Paula Singmann, Cornelia Prehn, Jerzy Adamski, Harald Grallert, Thomas Illig, Rui Wang-Sattler, Thomas Reinehr (2013). Metabolomics reveals determinants of weight loss during lifestyle intervention in obese children. Metabolomics.

Associated OMIM IDs

212750 (Celiac disease)
266600 (Crohn's disease)
610247 (Eosinophilic esophagitis)
606054 (Propionic acidemia)
601665 (Obesity)

External Links

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022268

KNApSAcK ID

Not Available

Chemspider ID

164090

KEGG Compound ID

C03017

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

188824

PDB ID

Not Available

ChEBI ID

53210

Food Biomarker Ontology

Not Available

VMH ID

PCRN

MarkerDB ID

MDB00000262

Good Scents ID

Not Available

References

Synthesis Reference

Not Available

Material Safety Data Sheet (MSDS)

Not Available

General References

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Ferrara F, Bertelli A, Falchi M: Evaluation of carnitine, acetylcarnitine and isovalerylcarnitine on immune function and apoptosis. Drugs Exp Clin Res. 2005;31(3):109-14. [PubMed:16033249 ]
Vermeulen RC, Scholte HR: Exploratory open label, randomized study of acetyl- and propionylcarnitine in chronic fatigue syndrome. Psychosom Med. 2004 Mar-Apr;66(2):276-82. [PubMed:15039515 ]
Vernez L, Wenk M, Krahenbuhl S: Determination of carnitine and acylcarnitines in plasma by high-performance liquid chromatography/electrospray ionization ion trap tandem mass spectrometry. Rapid Commun Mass Spectrom. 2004;18(11):1233-8. [PubMed:15164354 ]
Golan R, Soffer Y, Katz S, Weissenberg R, Wasserzug O, Lewin LM: Carnitine and short-chain acylcarnitines in the lumen of the human male reproductive tract. Int J Androl. 1983 Aug;6(4):349-57. [PubMed:6618689 ]
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 ]
Leonard JV, Vijayaraghavan S, Walter JH: The impact of screening for propionic and methylmalonic acidaemia. Eur J Pediatr. 2003 Dec;162 Suppl 1:S21-4. Epub 2003 Oct 30. [PubMed:14586648 ]
Penn D, Schmidt-Sommerfeld E, Jakobs C, Bieber LL: Amniotic fluid propionylcarnitine in methylmalonic aciduria. J Inherit Metab Dis. 1987;10(4):376-82. [PubMed:3126357 ]
Di Donato S, Rimoldi M, Garavaglia B, Uziel G: Propionylcarnitine excretion in propionic and methylmalonic acidurias: a cause of carnitine deficiency. Clin Chim Acta. 1984 May 16;139(1):13-21. [PubMed:6723070 ]
Bertelli A, Conte A, Ronca G: L-propionyl carnitine protects erythrocytes and low density lipoproteins against peroxidation. Drugs Exp Clin Res. 1994;20(5):191-7. [PubMed:7875055 ]
Van Hove JL, Chace DH, Kahler SG, Millington DS: Acylcarnitines in amniotic fluid: application to the prenatal diagnosis of propionic acidaemia. J Inherit Metab Dis. 1993;16(2):361-7. [PubMed:8411998 ]
Lucke T, Perez-Cerda C, Baumgartner M, Fowler B, Sander S, Sasse M, Scholl S, Ugarte M, Das AM: Propionic acidemia: unusual course with late onset and fatal outcome. Metabolism. 2004 Jun;53(6):809-10. [PubMed:15164333 ]
Zordoky BN, Sung MM, Ezekowitz J, Mandal R, Han B, Bjorndahl TC, Bouatra S, Anderson T, Oudit GY, Wishart DS, Dyck JR: Metabolomic fingerprint of heart failure with preserved ejection fraction. PLoS One. 2015 May 26;10(5):e0124844. doi: 10.1371/journal.pone.0124844. eCollection 2015. [PubMed:26010610 ]
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 ]
Cho K, Moon JS, Kang JH, Jang HB, Lee HJ, Park SI, Yu KS, Cho JY: Combined untargeted and targeted metabolomic profiling reveals urinary biomarkers for discriminating obese from normal-weight adolescents. Pediatr Obes. 2017 Apr;12(2):93-101. doi: 10.1111/ijpo.12114. Epub 2016 Feb 22. [PubMed:26910390 ]
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 ]
Kiykim E, Aktuglu Zeybek AC, Barut K, Zubarioglu T, Cansever MS, Alsancak S, Kasapcopur O: Screening of Free Carnitine and Acylcarnitine Status in Children With Familial Mediterranean Fever. Arch Rheumatol. 2016 Mar 10;31(2):133-138. doi: 10.5606/ArchRheumatol.2016.5696. eCollection 2016 Jun. [PubMed:29900937 ]
Adams SH, Hoppel CL, Lok KH, Zhao L, Wong SW, Minkler PE, Hwang DH, Newman JW, Garvey WT: Plasma acylcarnitine profiles suggest incomplete long-chain fatty acid beta-oxidation and altered tricarboxylic acid cycle activity in type 2 diabetic African-American women. J Nutr. 2009 Jun;139(6):1073-81. doi: 10.3945/jn.108.103754. Epub 2009 Apr 15. [PubMed:19369366 ]
Ueland T, Svardal A, Oie E, Askevold ET, Nymoen SH, Bjorndal B, Dahl CP, Gullestad L, Berge RK, Aukrust P: Disturbed carnitine regulation in chronic heart failure--increased plasma levels of palmitoyl-carnitine are associated with poor prognosis. Int J Cardiol. 2013 Sep 1;167(5):1892-9. doi: 10.1016/j.ijcard.2012.04.150. Epub 2012 May 22. [PubMed:22622056 ]
Han LS, Huang Z, Han F, Ye J, Qiu WJ, Zhang HW, Wang Y, Gong ZW, Gu XF: Clinical features and MUT gene mutation spectrum in Chinese patients with isolated methylmalonic acidemia: identification of ten novel allelic variants. World J Pediatr. 2015 Nov;11(4):358-65. doi: 10.1007/s12519-015-0043-1. Epub 2015 Oct 11. [PubMed:26454439 ]
Ghoraba DA, Mohammed MM, Zaki OK: Mutation analysis of methylmalonyl CoA mutase gene exon 2 in Egyptian families: Identification of 25 novel allelic variants. Meta Gene. 2015 Feb 25;3:71-88. doi: 10.1016/j.mgene.2014.02.001. eCollection 2015 Feb. [PubMed:25750861 ]
Kang L, Liu Y, Shen M, Liu Y, He R, Song J, Jin Y, Li M, Zhang Y, Dong H, Liu X, Yan H, Qin J, Zheng H, Chen Y, Li D, Wei H, Zhang H, Sun L, Zhu Z, Liang D, Yang Y: A study on a cohort of 301 Chinese patients with isolated methylmalonic acidemia. J Inherit Metab Dis. 2020 May;43(3):409-423. doi: 10.1002/jimd.12183. Epub 2019 Nov 26. [PubMed:31622506 ]
Keyfi F, Abbaszadegan MR, Sankian M, Rolfs A, Orolicki S, Pournasrollah M, Alijanpour M, Varasteh A: Mutation analysis of genes related to methylmalonic acidemia: identification of eight novel mutations. Mol Biol Rep. 2019 Feb;46(1):271-285. doi: 10.1007/s11033-018-4469-0. Epub 2019 Feb 2. [PubMed:30712249 ]
Curnock R, Heaton ND, Vilca-Melendez H, Dhawan A, Hadzic N, Vara R: Liver Transplantation in Children With Propionic Acidemia: Medium-Term Outcomes. Liver Transpl. 2020 Mar;26(3):419-430. doi: 10.1002/lt.25679. Epub 2020 Feb 3. [PubMed:31715057 ]
Monostori P, Klinke G, Richter S, Barath A, Fingerhut R, Baumgartner MR, Kolker S, Hoffmann GF, Gramer G, Okun JG: Simultaneous determination of 3-hydroxypropionic acid, methylmalonic acid and methylcitric acid in dried blood spots: Second-tier LC-MS/MS assay for newborn screening of propionic acidemia, methylmalonic acidemias and combined remethylation disorders. PLoS One. 2017 Sep 15;12(9):e0184897. doi: 10.1371/journal.pone.0184897. eCollection 2017. [PubMed:28915261 ]
Libert DM, Nowacki AS, Natowicz MR: Metabolomic analysis of obesity, metabolic syndrome, and type 2 diabetes: amino acid and acylcarnitine levels change along a spectrum of metabolic wellness. PeerJ. 2018 Aug 31;6:e5410. doi: 10.7717/peerj.5410. eCollection 2018. [PubMed:30186675 ]
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Gunstone, Frank D., John L. Harwood, and Albert J. Dijkstra (2007). The lipid handbook with CD-ROM. CRC Press.

Enzymes

Enzyme Details

1. Carnitine O-acetyltransferase

General function:: Involved in acyltransferase activity
Specific function:: Carnitine acetylase is specific for short chain fatty acids. Carnitine acetylase seems to affect the flux through the pyruvate dehydrogenase complex. It may be involved as well in the transport of acetyl-CoA into mitochondria.
Gene Name:: CRAT
Uniprot ID:: P43155
Molecular weight:: 70875.095

Enzyme Details

2. Mitochondrial carnitine/acylcarnitine carrier protein

General function:: Involved in transporter activity
Specific function:: Mediates the transport of acylcarnitines of different length across the mitochondrial inner membrane from the cytosol to the mitochondrial matrix for their oxidation by the mitochondrial fatty acid-oxidation pathway.
Gene Name:: SLC25A20
Uniprot ID:: O43772
Molecular weight:: 32943.46

Showing metabocard for Propionylcarnitine (HMDB0000824)

MOL for HMDB0000824 (Propionylcarnitine)

3D MOL for HMDB0000824 (Propionylcarnitine)

3D SDF for HMDB0000824 (Propionylcarnitine)

3D-SDF for HMDB0000824 (Propionylcarnitine)

PDB for HMDB0000824 (Propionylcarnitine)

3D PDB for HMDB0000824 (Propionylcarnitine)

SMILES for HMDB0000824 (Propionylcarnitine)

INCHI for HMDB0000824 (Propionylcarnitine)

Structure for HMDB0000824 (Propionylcarnitine)

3D Structure for HMDB0000824 (Propionylcarnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

Enzymes