You are using an unsupported browser. Please upgrade your browser to a newer version to get the best experience on Human Metabolome Database.

Jump To Section:

Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References XML

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

HMDB0000552

Secondary Accession Numbers

HMDB00552

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 (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. 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 (PMID: 35710135 ).

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>
HMDB0000552

> <DATABASE_NAME>
hmdb

> <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

View in JSmol View NMR Assignments View Stereo Labels

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

Chemical Formula

C₁₃H₂₃NO₆

Average Molecular Weight

289.328

Monoisotopic Molecular Weight

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

102673-95-0

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

Ontology

Physiological effect

Health effect

Cancer

Breast cancer (PMID: 35710135)

Diabetes mellitus type 2 (PMID: 35710135)
Celiac disease (PMID: 35710135)
Inflammatory bowel disease (PMID: 35710135)

Circulatory system disorder

Cardiac disorder

Cardiovascular disease (PMID: 35710135)
Heart failure (PMID: 35710135)

Renal system and urinary system

Renal disorder

Renal cell carcinoma (PMID: 35710135)

Disposition

Biological location

Cell membrane (HMDB: HMDB0000552)

Biofluid or Excreta

Non-excretory biofluid

Biofluid or Excreta

Blood (PMID: 24704061)
Saliva (HMDB: HMDB0000552)

Excreta

Biofluid or Excreta

Urine (PMID: 24023812)
Feces (PMID: 27624970)

Cellular substructure

Organelle

peroxisome (PMID: 35710135)

Cell

All cells and tissues (PMID: 35710135)

Route of exposure

Enteral

Ingestion (HMDB: HMDB0000552)

Source

Endogenous

Endogenous (HMDB: HMDB0000552)

Animal

Animal (HMDB: HMDB0000552)

Exogenous

Food

Food (HMDB: HMDB0000552)

Animal origin

Poultry

Greylag goose (FooDB: FOOD00368)
Guinea hen (FooDB: FOOD00373)
Ostrich (FooDB: FOOD00422)
Velvet duck (FooDB: FOOD00427)
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)

Equine

Horse (FooDB: FOOD00378)

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)

Milk and milk product

Unfermented milk

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)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid peroxidation (HMDB: HMDB0000552)

Biochemical process

Fatty acid metabolism (HMDB: HMDB0000552)

Cellular process

Cell signaling (HMDB: HMDB0000552)

Biochemical pathway

Lipid metabolism pathway (HMDB: HMDB0000552)

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	0.066 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

Predicted Chromatographic Properties

Predicted Collision Cross Sections

Predictor	Adduct Type	CCS Value (Å²)	Reference
DeepCCS	[M+H]+	164.656	30932474
DeepCCS	[M-H]-	160.827	30932474
DeepCCS	[M-2H]-	197.201	30932474
DeepCCS	[M+Na]+	173.219	30932474

Predicted Kovats Retention Indices

Underivatized

Metabolite	SMILES	Kovats RI Value	Column Type	Reference
3-Methylglutarylcarnitine	C[C@H](CC(O)=O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	3081.0	Standard polar	33892256
3-Methylglutarylcarnitine	C[C@H](CC(O)=O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1521.9	Standard non polar	33892256
3-Methylglutarylcarnitine	C[C@H](CC(O)=O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C	1968.0	Semi standard non polar	33892256

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
3-Methylglutarylcarnitine,1TMS,isomer #1	C[C@@H](CC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C)CC(=O)O[Si](C)(C)C	1954.9	Semi standard non polar	33892256
3-Methylglutarylcarnitine,1TBDMS,isomer #1	C[C@@H](CC(=O)O[C@H](CC(=O)[O-])C[N+](C)(C)C)CC(=O)O[Si](C)(C)C(C)(C)C	2193.3	Semi standard non polar	33892256

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutarylcarnitine GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutarylcarnitine GC-MS (TMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - 3-Methylglutarylcarnitine GC-MS (TBDMS_1_1) - 70eV, Positive	Not Available	2021-11-05	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutarylcarnitine 10V, Positive-QTOF	splash10-0006-0090000000-08d94c016258f3f2a373	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutarylcarnitine 20V, Positive-QTOF	splash10-000l-9050000000-42bdebd13e27d1118cb2	2021-09-22	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - 3-Methylglutarylcarnitine 40V, Positive-QTOF	splash10-000i-9000000000-e9262cbaff8cb4ad0ba6	2021-09-22	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-24	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm
Extracellular
Membrane

Biospecimen Locations

Blood
Feces
Saliva
Urine

Tissue Locations

Not Available

Pathways

Not Available

Name	SMPDB/PathBank	KEGG

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.0000-0.140 uM	Not Specified	Not Specified	Normal	23705938	details
Blood	Expected but not Quantified	Not Quantified	Not Available	Not Available	Normal	Not Available	details
Blood	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Normal	32966057	details
Blood	Detected and Quantified	0.020-0.060 uM	Adult (>18 years old)	Both	Normal	Molecular You	details
Feces	Detected and Quantified	0.24 +/- 0.1 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Feces	Detected and Quantified	1.1 +/- 0.65 nmol/g wet feces	Adult (>18 years old)	Both	Normal	27624970	details
Saliva	Detected and Quantified	0.088 +/- 0.011 uM	Adult (>18 years old)	Both	Normal	Zerihun T. Dame, ...	details
Urine	Detected and Quantified	0.03-0.13 umol/mmol creatinine	Newborn (0-30 days old)	Both	Normal	32340350	details
Urine	Detected and Quantified	0.05(0.03-0.13) umol/mmol creatinine	Newborn (0-30 days old)	Female	Normal	32340350	details
Urine	Detected and Quantified	0.06(0.03-0.13) 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	0.030 (0.016-0.052) umol/mmol creatinine	Adult (>18 years old)	Both	Normal	24023812	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.76 uM	Newborn (0-30 days old)	Male	3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency	23705938	details
Blood	Detected and Quantified	0.049 (0.0327) uM	Adult (>18 years old)	Female	Pregnancy with fetus having congenital heart defect	24704061	details

Associated Disorders and Diseases

Disease References

3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]

Associated OMIM IDs

246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)

External Links

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

VMH ID

Not Available

MarkerDB ID

MDB00029840

Good Scents ID

Not Available

References

Synthesis Reference

Jooste, S.; Erasmus, E.; Mienie, L. J.; de Wet, W. J.; Gibson, K. M. The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clinica Chimica Acta (1994), 230(1), 1-8.

Material Safety Data Sheet (MSDS)

Not Available

General References

Ferdinandusse S, Mulders J, IJlst L, Denis S, Dacremont G, Waterham HR, Wanders RJ: Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids. Biochem Biophys Res Commun. 1999 Sep 16;263(1):213-8. [PubMed:10486279 ]
Roe CR, Millington DS, Maltby DA: Identification of 3-methylglutarylcarnitine. A new diagnostic metabolite of 3-hydroxy-3-methylglutaryl-coenzyme A lyase deficiency. J Clin Invest. 1986 Apr;77(4):1391-4. [PubMed:3958190 ]
Lee C, Tsai FJ, Wu JY, Peng CT, Tsai CH, Hwu WL, Wang TR, Millington DS: 3-hydroxy-3-methylglutaric aciduria presenting with Reye like syndrome: report of one case. Acta Paediatr Taiwan. 1999 Nov-Dec;40(6):445-7. [PubMed:10927963 ]
Jooste S, Erasmus E, Mienie LJ, de Wet WJ, Gibson KM: The detection of 3-methylglutarylcarnitine and a new dicarboxylic conjugate, 3-methylglutaconylcarnitine, in 3-methylglutaconic aciduria. Clin Chim Acta. 1994 Oct 14;230(1):1-8. [PubMed:7850987 ]
Caksen H, Tuncer O, Kirimi E, Fryns JP, Uner A, Unal O, Cinal A, Odabas D: Report of two Turkish infants with Norman-Roberts syndrome. Genet Couns. 2004;15(1):9-17. [PubMed:15083694 ]
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. [PubMed:1635814 ]
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 ]
Bennett MJ, Coates PM, Hale DE, Millington DS, Pollitt RJ, Rinaldo P, Roe CR, Tanaka K: Analysis of abnormal urinary metabolites in the newborn period in medium-chain acyl-CoA dehydrogenase deficiency. J Inherit Metab Dis. 1990;13(5):707-15. [PubMed:2246856 ]
Fontaine M, Briand G, Largilliere C, Degand P, Divry P, Vianey-Saban C, Mousson B, Vamecq J: Metabolic studies in a patient with severe carnitine palmitoyltransferase type II deficiency. Clin Chim Acta. 1998 May 25;273(2):161-70. [PubMed:9657346 ]
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 ]
Violante S, Achetib N, van Roermund CWT, Hagen J, Dodatko T, Vaz FM, Waterham HR, Chen H, Baes M, Yu C, Argmann CA, Houten SM: Peroxisomes can oxidize medium- and long-chain fatty acids through a pathway involving ABCD3 and HSD17B4. FASEB J. 2019 Mar;33(3):4355-4364. doi: 10.1096/fj.201801498R. Epub 2018 Dec 12. [PubMed:30540494 ]
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 ]
Chen C, Hou G, Zeng C, Ren Y, Chen X, Peng C: Metabolomic profiling reveals amino acid and carnitine alterations as metabolic signatures in psoriasis. Theranostics. 2021 Jan 1;11(2):754-767. doi: 10.7150/thno.51154. eCollection 2021. [PubMed:33391503 ]
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 ]
Mihalik SJ, Goodpaster BH, Kelley DE, Chace DH, Vockley J, Toledo FG, DeLany JP: Increased levels of plasma acylcarnitines in obesity and type 2 diabetes and identification of a marker of glucolipotoxicity. Obesity (Silver Spring). 2010 Sep;18(9):1695-700. doi: 10.1038/oby.2009.510. Epub 2010 Jan 28. [PubMed:20111019 ]
Zhao S, Feng XF, Huang T, Luo HH, Chen JX, Zeng J, Gu M, Li J, Sun XY, Sun D, Yang X, Fang ZZ, Cao YF: The Association Between Acylcarnitine Metabolites and Cardiovascular Disease in Chinese Patients With Type 2 Diabetes Mellitus. Front Endocrinol (Lausanne). 2020 May 5;11:212. doi: 10.3389/fendo.2020.00212. eCollection 2020. [PubMed:32431666 ]
Saral NY, Aksungar FB, Aktuglu-Zeybek C, Coskun J, Demirelce O, Serteser M: Glutaric acidemia type II patient with thalassemia minor and novel electron transfer flavoprotein-A gene mutations: A case report and review of literature. World J Clin Cases. 2018 Nov 26;6(14):786-790. doi: 10.12998/wjcc.v6.i14.786. [PubMed:30510944 ]
Kukharenko A, Brito A, Kozhevnikova MV, Moskaleva N, Markin PA, Bochkareva N, Korobkova EO, Belenkov YN, Privalova EV, Larcova EV, Ariani A, La Frano MR, Appolonova SA: Relationship between the plasma acylcarnitine profile and cardiometabolic risk factors in adults diagnosed with cardiovascular diseases. Clin Chim Acta. 2020 Aug;507:250-256. doi: 10.1016/j.cca.2020.04.035. Epub 2020 May 4. [PubMed:32376321 ]
Batchuluun B, Al Rijjal D, Prentice KJ, Eversley JA, Burdett E, Mohan H, Bhattacharjee A, Gunderson EP, Liu Y, Wheeler MB: Elevated Medium-Chain Acylcarnitines Are Associated With Gestational Diabetes Mellitus and Early Progression to Type 2 Diabetes and Induce Pancreatic beta-Cell Dysfunction. Diabetes. 2018 May;67(5):885-897. doi: 10.2337/db17-1150. Epub 2018 Feb 7. [PubMed:29436377 ]
Dambrova M, Makrecka-Kuka M, Kuka J, Vilskersts R, Nordberg D, Attwood MM, Smesny S, Sen ZD, Guo AC, Oler E, Tian S, Zheng J, Wishart DS, Liepinsh E, Schioth HB: Acylcarnitines: Nomenclature, Biomarkers, Therapeutic Potential, Drug Targets, and Clinical Trials. Pharmacol Rev. 2022 Jul;74(3):506-551. doi: 10.1124/pharmrev.121.000408. [PubMed:35710135 ]

Showing metabocard for 3-Methylglutarylcarnitine (HMDB0000552)

MOL for HMDB0000552 (3-Methylglutarylcarnitine)

3D MOL for HMDB0000552 (3-Methylglutarylcarnitine)

3D SDF for HMDB0000552 (3-Methylglutarylcarnitine)

3D-SDF for HMDB0000552 (3-Methylglutarylcarnitine)

PDB for HMDB0000552 (3-Methylglutarylcarnitine)

3D PDB for HMDB0000552 (3-Methylglutarylcarnitine)

SMILES for HMDB0000552 (3-Methylglutarylcarnitine)

INCHI for HMDB0000552 (3-Methylglutarylcarnitine)

Structure for HMDB0000552 (3-Methylglutarylcarnitine)

3D Structure for HMDB0000552 (3-Methylglutarylcarnitine)

Predicted Collision Cross Sections

Predicted Kovats Retention Indices

Underivatized

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra