MiMeDB: Showing metabocard for 3-Methylglutarylcarnitine (MMDBc0000526)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2020-12-10 18:39:46 UTC

Update Date

2024-04-30 19:32:48 UTC

Metabolite ID

MMDBc0000526

Metabolite Identification

Common Name

3-Methylglutarylcarnitine

Description

3-Methylglutarylcarnitine is an acylcarnitine. More specifically, it is an methylglutaric 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 [Dambrova et al. 2021, Physiological Reviews], 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. 3-Methylglutarylcarnitine is therefore classified as a medium chain AC. As a medium-chain acylcarnitine 3-methylglutarylcarnitine is somewhat less abundant than short-chain acylcarnitines. These are formed either through esterification with L-carnitine or through the peroxisomal metabolism of longer chain acylcarnitines (PMID: 30540494 ). Many medium-chain acylcarnitines can serve as useful markers for inherited disorders of fatty acid metabolism. In particular 3-methylglutarylcarnitine is elevated in the blood or plasma of individuals with psoriasis (PMID: 33391503 ), CVD (PMID: 32376321 ), Norman-Roberts syndrome (PMID: 15083694 ), type 2 diabetes Mellitus (PMID: 20111019 , PMID: 19369366 , PMID: 29436377 ), carnitine palmitoyl-trasferase 2 deficiency (PMID: 9657346 ), Familial Mediterranean Fever (PMID: 29900937 ), multiple acyl coenzyme A dehydrogenase Deficiency (PMID: 30510944 ), CVD in type 2 diabetes Mellitus (PMID: 32431666 ), and gestational diabetes mellitus (PMID: 29436377 ). It is also decreased in the blood or plasma of individuals with Celiac disease (PMID: 16425363 ). 3-Methylglutarylcarnitine is elevated in the urine of individuals with medium-chain acyl-CoA dehydrogenase deficiency (PMID: 1635814 , PMID: 2246856 ). 3-Methylglutarylcarnitine is a diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. It is also identified in the urine of patients with Reye-like syndrome (PMID: 3958190 , 10927963 ). Carnitine octanoyltransferase (CrOT, EC:2.3.1.137) is responsible for the synthesis of all medium-chain (MCAC, C5-C12) and medium-length branched-chain acylcarnitines in peroxisomes (PMID: 10486279 ). 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 at [Dambrova et al. 2021, Physiological Reviews].

Structure

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

HMDB0000552
     RDKit          3D
3-Methylglutarylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    2.8939   -1.7972   -0.7186 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1694   -0.3584   -1.1302 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.9444    0.3193   -0.0419 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2823    1.7217   -0.3146 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9481    2.2886   -1.3784 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.9914    2.4525    0.6234 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8421    0.2802   -1.4353 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9173    0.2877   -0.2703 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9844    1.1284    0.6528 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0982   -0.6418   -0.1152 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9806   -0.6338    0.9930 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8665   -1.9118    1.7928 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7753   -1.8284    2.9553 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4318   -0.8000    3.2557 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9641   -2.9180    3.8138 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3571   -0.5716    0.4050 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6683    0.5633   -0.4037 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.9018    0.7513   -1.5860 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0792    0.3604   -0.8297 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5905    1.7874    0.3838 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0816   -2.2468   -1.3271 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8225   -2.3629   -0.9684 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6764   -1.8773    0.3588 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7323   -0.4009   -2.0818 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8732   -0.3086    0.1026 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3687    0.1884    0.9171 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.5771    2.5485    1.5563 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3791   -0.3807   -2.2288 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.9395    1.2664   -1.9234 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8312    0.1838    1.7021 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1728   -2.0013    2.2230 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1292   -2.8108    1.2124 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1604   -0.6548    1.1598 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4069   -1.4931   -0.2737 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3828   -0.1333   -1.9747 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6226    1.0240   -2.4179 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2531    1.6653   -1.5356 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.7165    0.9514   -0.1415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2317    0.7728   -1.8390 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3307   -0.7162   -0.8229 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4836    1.7818    1.0415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6396    1.8409    0.9066 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6949    2.6837   -0.2973 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
  4  5  2  0
  4  6  1  0
  2  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
 10 11  1  0
 11 12  1  0
 12 13  1  0
 13 14  2  0
 13 15  1  0
 11 16  1  0
 16 17  1  0
 17 18  1  0
 17 19  1  0
 17 20  1  0
  1 21  1  0
  1 22  1  0
  1 23  1  0
  2 24  1  6
  3 25  1  0
  3 26  1  0
  6 27  1  0
  7 28  1  0
  7 29  1  0
 11 30  1  1
 12 31  1  0
 12 32  1  0
 16 33  1  0
 16 34  1  0
 18 35  1  0
 18 36  1  0
 18 37  1  0
 19 38  1  0
 19 39  1  0
 19 40  1  0
 20 41  1  0
 20 42  1  0
 20 43  1  0
M  CHG  2  15  -1  17   1
M  END


  Mrv1652303232018532D          

 20 19  0  0  0  0            999 V2000
 2493.9443 2499.8005    0.0000 C   0  0  1  0  0  0  0  0  0  0  0  0
 2494.6583 2499.3872    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 2495.3723 2499.8005    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2496.0863 2499.3872    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2495.3723 2500.6248    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 2493.9443 2500.6248    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2493.2303 2501.0360    0.0000 N   0  3  0  0  0  0  0  0  0  0  0  0
 2492.5122 2500.6230    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2493.2303 2501.8607    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2492.3773 2501.5292    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2493.2365 2499.3769    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2493.2365 2498.5526    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2493.9526 2498.1392    0.0000 O   0  5  0  0  0  0  0  0  0  0  0  0
 2492.5246 2498.1392    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 2496.8012 2499.7988    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
 2496.8021 2500.6238    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2497.5152 2499.3855    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2498.2301 2499.7972    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
 2498.2311 2500.6222    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
 2498.9441 2499.3839    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0  0  0  0
  1  6  1  6  0  0  0
  1 11  1  0  0  0  0
  2  3  1  0  0  0  0
  3  4  1  0  0  0  0
  3  5  2  0  0  0  0
  6  7  1  0  0  0  0
  7  8  1  0  0  0  0
  7  9  1  0  0  0  0
  7 10  1  0  0  0  0
 11 12  1  0  0  0  0
 12 13  1  0  0  0  0
 12 14  2  0  0  0  0
  4 15  1  0  0  0  0
 15 16  1  6  0  0  0
 15 17  1  0  0  0  0
 17 18  1  0  0  0  0
 18 19  2  0  0  0  0
 18 20  1  0  0  0  0
M  CHG  2   7   1  13  -1
M  END
> <DATABASE_ID>
MMDBc0000526

> <DATABASE_NAME>
MIME

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

> <INCHI_IDENTIFIER>
InChI=1S/C13H23NO6/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18)/t9-,10-/m1/s1

> <INCHI_KEY>
HFCPFJNSBPQJDP-NXEZZACHSA-N

> <FORMULA>
C13H23NO6

> <MOLECULAR_WEIGHT>
289.328

> <EXACT_MASS>
289.152537465

> <JCHEM_ACCEPTOR_COUNT>
5

> <JCHEM_ATOM_COUNT>
43

> <JCHEM_AVERAGE_POLARIZABILITY>
29.55161809088242

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
1

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

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

> <ALOGPS_LOGP>
-1.75

> <JCHEM_LOGP>
-3.889930129471746

> <ALOGPS_LOGS>
-3.73

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
0

> <JCHEM_PHYSIOLOGICAL_CHARGE>
-1

> <JCHEM_PKA>
4.283411821813018

> <JCHEM_PKA_STRONGEST_ACIDIC>
3.4232354107940064

> <JCHEM_PKA_STRONGEST_BASIC>
-7.060302232707195

> <JCHEM_POLAR_SURFACE_AREA>
103.73

> <JCHEM_REFRACTIVITY>
92.67969999999998

> <JCHEM_ROTATABLE_BOND_COUNT>
10

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
6.43e-02 g/l

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

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000552
     RDKit          3D
3-Methylglutarylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    2.8939   -1.7972   -0.7186 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1694   -0.3584   -1.1302 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.9444    0.3193   -0.0419 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2823    1.7217   -0.3146 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9481    2.2886   -1.3784 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.9914    2.4525    0.6234 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8421    0.2802   -1.4353 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9173    0.2877   -0.2703 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9844    1.1284    0.6528 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0982   -0.6418   -0.1152 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9806   -0.6338    0.9930 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8665   -1.9118    1.7928 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7753   -1.8284    2.9553 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4318   -0.8000    3.2557 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9641   -2.9180    3.8138 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3571   -0.5716    0.4050 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6683    0.5633   -0.4037 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.9018    0.7513   -1.5860 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0792    0.3604   -0.8297 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5905    1.7874    0.3838 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0816   -2.2468   -1.3271 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8225   -2.3629   -0.9684 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6764   -1.8773    0.3588 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7323   -0.4009   -2.0818 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8732   -0.3086    0.1026 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3687    0.1884    0.9171 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.5771    2.5485    1.5563 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3791   -0.3807   -2.2288 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.9395    1.2664   -1.9234 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8312    0.1838    1.7021 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1728   -2.0013    2.2230 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1292   -2.8108    1.2124 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1604   -0.6548    1.1598 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4069   -1.4931   -0.2737 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3828   -0.1333   -1.9747 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6226    1.0240   -2.4179 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2531    1.6653   -1.5356 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.7165    0.9514   -0.1415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2317    0.7728   -1.8390 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3307   -0.7162   -0.8229 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4836    1.7818    1.0415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6396    1.8409    0.9066 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6949    2.6837   -0.2973 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
  4  5  2  0
  4  6  1  0
  2  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
 10 11  1  0
 11 12  1  0
 12 13  1  0
 13 14  2  0
 13 15  1  0
 11 16  1  0
 16 17  1  0
 17 18  1  0
 17 19  1  0
 17 20  1  0
  1 21  1  0
  1 22  1  0
  1 23  1  0
  2 24  1  6
  3 25  1  0
  3 26  1  0
  6 27  1  0
  7 28  1  0
  7 29  1  0
 11 30  1  1
 12 31  1  0
 12 32  1  0
 16 33  1  0
 16 34  1  0
 18 35  1  0
 18 36  1  0
 18 37  1  0
 19 38  1  0
 19 39  1  0
 19 40  1  0
 20 41  1  0
 20 42  1  0
 20 43  1  0
M  CHG  2  15  -1  17   1
M  END

HEADER    PROTEIN                                 23-MAR-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   23-MAR-20         0                                  
HETATM    1  C   UNK     0    4655.3634666.294   0.000  0.00  0.00           C+0
HETATM    2  O   UNK     0    4656.6954665.523   0.000  0.00  0.00           O+0
HETATM    3  C   UNK     0    4658.0284666.294   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    4659.3614665.523   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    4658.0284667.833   0.000  0.00  0.00           O+0
HETATM    6  C   UNK     0    4655.3634667.833   0.000  0.00  0.00           C+0
HETATM    7  N   UNK     0    4654.0304668.601   0.000  0.00  0.00           N+1
HETATM    8  C   UNK     0    4652.6894667.830   0.000  0.00  0.00           C+0
HETATM    9  C   UNK     0    4654.0304670.140   0.000  0.00  0.00           C+0
HETATM   10  C   UNK     0    4652.4384669.521   0.000  0.00  0.00           C+0
HETATM   11  C   UNK     0    4654.0414665.504   0.000  0.00  0.00           C+0
HETATM   12  C   UNK     0    4654.0414663.965   0.000  0.00  0.00           C+0
HETATM   13  O   UNK     0    4655.3784663.193   0.000  0.00  0.00           O-1
HETATM   14  O   UNK     0    4652.7134663.193   0.000  0.00  0.00           O+0
HETATM   15  C   UNK     0    4660.6964666.291   0.000  0.00  0.00           C+0
HETATM   16  C   UNK     0    4660.6974667.831   0.000  0.00  0.00           C+0
HETATM   17  C   UNK     0    4662.0284665.520   0.000  0.00  0.00           C+0
HETATM   18  C   UNK     0    4663.3634666.288   0.000  0.00  0.00           C+0
HETATM   19  O   UNK     0    4663.3654667.828   0.000  0.00  0.00           O+0
HETATM   20  O   UNK     0    4664.6964665.517   0.000  0.00  0.00           O+0
CONECT    1    2    6   11                                                  
CONECT    2    1    3                                                       
CONECT    3    2    4    5                                                  
CONECT    4    3   15                                                       
CONECT    5    3                                                            
CONECT    6    1    7                                                       
CONECT    7    6    8    9   10                                             
CONECT    8    7                                                            
CONECT    9    7                                                            
CONECT   10    7                                                            
CONECT   11    1   12                                                       
CONECT   12   11   13   14                                                  
CONECT   13   12                                                            
CONECT   14   12                                                            
CONECT   15    4   16   17                                                  
CONECT   16   15                                                            
CONECT   17   15   18                                                       
CONECT   18   17   19   20                                                  
CONECT   19   18                                                            
CONECT   20   18                                                            
MASTER        0    0    0    0    0    0    0    0   20    0   38    0
END

COMPND    HMDB0000552
HETATM    1  C1  UNL     1       2.894  -1.797  -0.719  1.00  0.00           C  
HETATM    2  C2  UNL     1       3.169  -0.358  -1.130  1.00  0.00           C  
HETATM    3  C3  UNL     1       3.944   0.319  -0.042  1.00  0.00           C  
HETATM    4  C4  UNL     1       4.282   1.722  -0.315  1.00  0.00           C  
HETATM    5  O1  UNL     1       3.948   2.289  -1.378  1.00  0.00           O  
HETATM    6  O2  UNL     1       4.991   2.452   0.623  1.00  0.00           O  
HETATM    7  C5  UNL     1       1.842   0.280  -1.435  1.00  0.00           C  
HETATM    8  C6  UNL     1       0.917   0.288  -0.270  1.00  0.00           C  
HETATM    9  O3  UNL     1       0.984   1.128   0.653  1.00  0.00           O  
HETATM   10  O4  UNL     1      -0.098  -0.642  -0.115  1.00  0.00           O  
HETATM   11  C7  UNL     1      -0.981  -0.634   0.993  1.00  0.00           C  
HETATM   12  C8  UNL     1      -0.866  -1.912   1.793  1.00  0.00           C  
HETATM   13  C9  UNL     1      -1.775  -1.828   2.955  1.00  0.00           C  
HETATM   14  O5  UNL     1      -2.432  -0.800   3.256  1.00  0.00           O  
HETATM   15  O6  UNL     1      -1.964  -2.918   3.814  1.00  0.00           O1-
HETATM   16  C10 UNL     1      -2.357  -0.572   0.405  1.00  0.00           C  
HETATM   17  N1  UNL     1      -2.668   0.563  -0.404  1.00  0.00           N1+
HETATM   18  C11 UNL     1      -1.902   0.751  -1.586  1.00  0.00           C  
HETATM   19  C12 UNL     1      -4.079   0.360  -0.830  1.00  0.00           C  
HETATM   20  C13 UNL     1      -2.591   1.787   0.384  1.00  0.00           C  
HETATM   21  H1  UNL     1       2.082  -2.247  -1.327  1.00  0.00           H  
HETATM   22  H2  UNL     1       3.823  -2.363  -0.968  1.00  0.00           H  
HETATM   23  H3  UNL     1       2.676  -1.877   0.359  1.00  0.00           H  
HETATM   24  H4  UNL     1       3.732  -0.401  -2.082  1.00  0.00           H  
HETATM   25  H5  UNL     1       4.873  -0.309   0.103  1.00  0.00           H  
HETATM   26  H6  UNL     1       3.369   0.188   0.917  1.00  0.00           H  
HETATM   27  H7  UNL     1       4.577   2.548   1.556  1.00  0.00           H  
HETATM   28  H8  UNL     1       1.379  -0.381  -2.229  1.00  0.00           H  
HETATM   29  H9  UNL     1       1.940   1.266  -1.923  1.00  0.00           H  
HETATM   30  H10 UNL     1      -0.831   0.184   1.702  1.00  0.00           H  
HETATM   31  H11 UNL     1       0.173  -2.001   2.223  1.00  0.00           H  
HETATM   32  H12 UNL     1      -1.129  -2.811   1.212  1.00  0.00           H  
HETATM   33  H13 UNL     1      -3.160  -0.655   1.160  1.00  0.00           H  
HETATM   34  H14 UNL     1      -2.407  -1.493  -0.274  1.00  0.00           H  
HETATM   35  H15 UNL     1      -1.383  -0.133  -1.975  1.00  0.00           H  
HETATM   36  H16 UNL     1      -2.623   1.024  -2.418  1.00  0.00           H  
HETATM   37  H17 UNL     1      -1.253   1.665  -1.536  1.00  0.00           H  
HETATM   38  H18 UNL     1      -4.716   0.951  -0.142  1.00  0.00           H  
HETATM   39  H19 UNL     1      -4.232   0.773  -1.839  1.00  0.00           H  
HETATM   40  H20 UNL     1      -4.331  -0.716  -0.823  1.00  0.00           H  
HETATM   41  H21 UNL     1      -3.484   1.782   1.041  1.00  0.00           H  
HETATM   42  H22 UNL     1      -1.640   1.841   0.907  1.00  0.00           H  
HETATM   43  H23 UNL     1      -2.695   2.684  -0.297  1.00  0.00           H  
CONECT    1    2   21   22   23
CONECT    2    3    7   24
CONECT    3    4   25   26
CONECT    4    5    5    6
CONECT    6   27
CONECT    7    8   28   29
CONECT    8    9    9   10
CONECT   10   11
CONECT   11   12   16   30
CONECT   12   13   31   32
CONECT   13   14   14   15
CONECT   16   17   33   34
CONECT   17   18   19   20
CONECT   18   35   36   37
CONECT   19   38   39   40
CONECT   20   41   42   43
END

HMDB0000552
     RDKit          3D
3-Methylglutarylcarnitine
 43 42  0  0  0  0  0  0  0  0999 V2000
    2.8939   -1.7972   -0.7186 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.1694   -0.3584   -1.1302 C   0  0  2  0  0  0  0  0  0  0  0  0
    3.9444    0.3193   -0.0419 C   0  0  0  0  0  0  0  0  0  0  0  0
    4.2823    1.7217   -0.3146 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.9481    2.2886   -1.3784 O   0  0  0  0  0  0  0  0  0  0  0  0
    4.9914    2.4525    0.6234 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.8421    0.2802   -1.4353 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9173    0.2877   -0.2703 C   0  0  0  0  0  0  0  0  0  0  0  0
    0.9844    1.1284    0.6528 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0982   -0.6418   -0.1152 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9806   -0.6338    0.9930 C   0  0  2  0  0  0  0  0  0  0  0  0
   -0.8665   -1.9118    1.7928 C   0  0  0  0  0  0  0  0  0  0  0  0
   -1.7753   -1.8284    2.9553 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4318   -0.8000    3.2557 O   0  0  0  0  0  0  0  0  0  0  0  0
   -1.9641   -2.9180    3.8138 O   0  0  0  0  0  1  0  0  0  0  0  0
   -2.3571   -0.5716    0.4050 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6683    0.5633   -0.4037 N   0  0  0  0  0  4  0  0  0  0  0  0
   -1.9018    0.7513   -1.5860 C   0  0  0  0  0  0  0  0  0  0  0  0
   -4.0792    0.3604   -0.8297 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.5905    1.7874    0.3838 C   0  0  0  0  0  0  0  0  0  0  0  0
    2.0816   -2.2468   -1.3271 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.8225   -2.3629   -0.9684 H   0  0  0  0  0  0  0  0  0  0  0  0
    2.6764   -1.8773    0.3588 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.7323   -0.4009   -2.0818 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.8732   -0.3086    0.1026 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.3687    0.1884    0.9171 H   0  0  0  0  0  0  0  0  0  0  0  0
    4.5771    2.5485    1.5563 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.3791   -0.3807   -2.2288 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.9395    1.2664   -1.9234 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.8312    0.1838    1.7021 H   0  0  0  0  0  0  0  0  0  0  0  0
    0.1728   -2.0013    2.2230 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.1292   -2.8108    1.2124 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1604   -0.6548    1.1598 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4069   -1.4931   -0.2737 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.3828   -0.1333   -1.9747 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6226    1.0240   -2.4179 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.2531    1.6653   -1.5356 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.7165    0.9514   -0.1415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.2317    0.7728   -1.8390 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.3307   -0.7162   -0.8229 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.4836    1.7818    1.0415 H   0  0  0  0  0  0  0  0  0  0  0  0
   -1.6396    1.8409    0.9066 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.6949    2.6837   -0.2973 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
  4  5  2  0
  4  6  1  0
  2  7  1  0
  7  8  1  0
  8  9  2  0
  8 10  1  0
 10 11  1  0
 11 12  1  0
 12 13  1  0
 13 14  2  0
 13 15  1  0
 11 16  1  0
 16 17  1  0
 17 18  1  0
 17 19  1  0
 17 20  1  0
  1 21  1  0
  1 22  1  0
  1 23  1  0
  2 24  1  6
  3 25  1  0
  3 26  1  0
  6 27  1  0
  7 28  1  0
  7 29  1  0
 11 30  1  1
 12 31  1  0
 12 32  1  0
 16 33  1  0
 16 34  1  0
 18 35  1  0
 18 36  1  0
 18 37  1  0
 19 38  1  0
 19 39  1  0
 19 40  1  0
 20 41  1  0
 20 42  1  0
 20 43  1  0
M  CHG  2  15  -1  17   1
M  END

Synonyms

Value	Source
(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid	HMDB
Methylglutarylcarnitine	HMDB
O-3-Methylglutarylcarnitine	HMDB
3-Methylglutarylcarnitine	HMDB

Molecular Formula

C₁₃H₂₃NO₆

Average Mass

289.328

Monoisotopic Mass

289.152537465

IUPAC Name

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

Traditional Name

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

CAS Registry Number

Not Available

SMILES

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

InChI Identifier

InChI=1S/C13H23NO6/c1-9(5-11(15)16)6-13(19)20-10(7-12(17)18)8-14(2,3)4/h9-10H,5-8H2,1-4H3,(H-,15,16,17,18)/t9-,10-/m1/s1

InChI Key

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

Not Available

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Property	Value	Source
Water Solubility	0.064 g/L	ALOGPS
logP	-1.8	ALOGPS
logP	-3.9	ChemAxon
logS	-3.7	ALOGPS
pKa (Strongest Acidic)	3.42	ChemAxon
pKa (Strongest Basic)	-7.1	ChemAxon
Physiological Charge	-1	ChemAxon
Hydrogen Acceptor Count	5	ChemAxon
Hydrogen Donor Count	1	ChemAxon
Polar Surface Area	103.73 Å²	ChemAxon
Rotatable Bond Count	10	ChemAxon
Refractivity	92.68 m³·mol⁻¹	ChemAxon
Polarizability	29.55 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

Spectra

Spectrum Type	Description	Splash Key	Deposition Date	View
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 10V, Positive	splash10-0006-0090000000-08d94c016258f3f2a373	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 20V, Positive	splash10-000l-9050000000-42bdebd13e27d1118cb2	2021-09-22	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 40V, Positive	splash10-000i-9000000000-e9262cbaff8cb4ad0ba6	2021-09-22	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum
1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)		2021-09-24	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane

Biospecimen Locations

Blood
Feces
Saliva
Urine

Tissue Locations

Not Available

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species

Human Pathways

Pathways

Name	SMPDB/PathBank

Metabolic Reactions

Not Available

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
3-hydroxy-3-methylglutaryl-coa lyase deficiency		Association	Human Blood	HMDB	23705938

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Bacteroidetes	2	Human feces; Human stool; Human	Indirect association between microbiota composition and metabolites measured in host biospecimen

Exposure Sources

Source Type	Source Sub-type	Species	Data Source	Reference

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Blood	Detected and Quantified	0.76				uM	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	HMDB	23705938
Human Blood	Detected and Quantified			0.0000	0.140	uM	Not Specified	Not Specified	Normal	HMDB	23705938
Human Urine	Detected and Quantified			0.03	0.13	umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	HMDB	32340350
Human Urine	Detected and Quantified	0.030		0.016	0.052	umol/mmol creatinine	Adult (>18 years old)	Both	Normal	HMDB	24023812
Human Urine	Detected and Quantified	0.05		0.03	0.13	umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	HMDB	32340350
Human Urine	Detected and Quantified	0.06		0.03	0.13	umol/mmol creatinine	Newborn (0-30 days old)	Male	Normal	HMDB	32340350
Human Feces	Detected and Quantified	0.24	0.1			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Feces	Detected and Quantified	1.1	0.65			nmol/g wet feces	Adult (>18 years old)	Both	Normal	HMDB	27624970
Human Saliva	Detected and Quantified	0.088	0.011			uM	Adult (>18 years old)	Both	Normal	HMDB	https://doi.org/10.1007/s11306-015-0840-5
Human Blood	Detected and Quantified			0.020	0.060	uM	Adult (>18 years old)	Both	Normal	HMDB	Molecular You (https://molecularyou.com)
Human Urine	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057
Human Blood	Detected but not Quantified						Adult (>18 years old)	Both	Normal	HMDB	32966057

External Links

HMDB ID

HMDB0000552

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022112

KNApSAcK ID

Not Available

Chemspider ID

Not Available

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

Not Available

PubChem Compound

Not Available

PDB ID

Not Available

ChEBI ID

Not Available

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

Schmidt-Sommerfeld E, Penn D, Rinaldo P, Kossak D, Li BU, Huang ZH, Gage DA: Urinary medium-chain acylcarnitines in medium-chain acyl-CoA dehydrogenase deficiency, medium-chain triglyceride feeding and valproic acid therapy: sensitivity and specificity of the radioisotopic exchange/high performance liquid chromatography method. Pediatr Res. 1992 Jun;31(6):545-51. doi: 10.1203/00006450-199206000-00002. [PubMed:1635814 ]
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Showing metabocard for 3-Methylglutarylcarnitine (MMDBc0000526)

MOL for #<Metabolite:0x000055e8c8d20eb8>

3D MOL for #<Metabolite:0x000055e8c8d20eb8>

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3D-SDF for #<Metabolite:0x000055e8c8d20eb8>

PDB for #<Metabolite:0x000055e8c8d20eb8>

3D PDB for #<Metabolite:0x000055e8c8d20eb8>

SMILES for #<Metabolite:0x000055e8c8d20eb8>

INCHI for #<Metabolite:0x000055e8c8d20eb8>

Structure for #<Metabolite:0x000055e8c8d20eb8>

3D Structure for #<Metabolite:0x000055e8c8d20eb8>