ChemFOnt: Showing chemical card for 3-Methylglutarylcarnitine (CFc000000428)

Record Information

Version

1.0

Creation Date

2022-08-28 10:25:12 UTC

Update Date

2022-09-13 18:44:49 UTC

Chemfont ID

CFc000000428

Molecule Identification

Common Name

3-Methylglutarylcarnitine

Definition

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 SDF PDB SMILES InChI

Synonyms

Value	Source
O-3-Methylglutarylcarnitine	HMDB
Methylglutaroylcarnitine	HMDB
Methylglutaryl-L-carnitine	HMDB
3-Methylglutarylcarnitine	MeSH

Chemical Formula

C₁₃H₂₃NO₆

Average Molecular Weight

289.3248

Monoisotopic Molecular Weight

289.152537473

IUPAC Name

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

Traditional Name

3-methylglutarylcarnitine

CAS Registry Number

102673-95-0

SMILES

CC(CC(O)=O)CC(=O)OC(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)

InChI Key

HFCPFJNSBPQJDP-UHFFFAOYSA-N

Chemical Taxonomy

Description

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

Kingdom

Organic compounds

Super Class

Lipids and lipid-like molecules

Class

Fatty Acyls

Sub Class

Fatty acid esters

Direct Parent

Acyl carnitines

Alternative Parents

Substituents

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

Molecular Framework

Aliphatic acyclic compounds

External Descriptors

O-methylglutarylcarnitine (CHEBI:70857 )

Functional Ontology

Physiological effect

Disposition

Biological location

Non-excretory biofluid

Biofluid or Excreta

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

Cellular substructure

Organelle

Peroxisome (PMID: 35710135)

Excreta

Biofluid or Excreta

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

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

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

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)

Exogenous (HMDB: HMDB0000552)

Process

Naturally occurring process

Biological process

Chemical reaction

Lipid Peroxidation (HMDB: HMDB0000552)

Biochemical pathway

Metabolic pathway

Fatty Acid Metabolism (HMDB: HMDB0000552)

Lipid metabolism pathway (HMDB: HMDB0000552)

Cellular process

Cell signaling (HMDB: HMDB0000552)

Biochemical process

Lipid transport (HMDB: HMDB0000552)

Role

Physical Properties

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	29.65 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

External Links

HMDB ID

HMDB0000552

DrugBank ID

Not Available

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB022112

KNApSAcK ID

Not Available

Chemspider ID

113619

KEGG Compound ID

Not Available

BioCyc ID

Not Available

BiGG ID

Not Available

Wikipedia Link

Not Available

METLIN ID

5536

PubChem Compound

128145

PDB ID

Not Available

ChEBI ID

70857

Food Biomarker Ontology

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.

Showing chemical card for 3-Methylglutarylcarnitine (CFc000000428)

MOL for CFc000000428 (3-Methylglutarylcarnitine)

3D MOL for CFc000000428 (3-Methylglutarylcarnitine)

3D SDF for CFc000000428 (3-Methylglutarylcarnitine)

3D-SDF for CFc000000428 (3-Methylglutarylcarnitine)

PDB for CFc000000428 (3-Methylglutarylcarnitine)

3D PDB for CFc000000428 (3-Methylglutarylcarnitine)

SMILES for CFc000000428 (3-Methylglutarylcarnitine)

INCHI for CFc000000428 (3-Methylglutarylcarnitine)

Structure for CFc000000428 (3-Methylglutarylcarnitine)

3D Structure for CFc000000428 (3-Methylglutarylcarnitine)