| Common Name |
3-Methylglutarylcarnitine
| Description |
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).
| Structure |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
Methylglutaryl-L-carnitineHMDB
| Chemical Formlia |
C13H23NO6
| Average Molecliar Weight |
289.3248
| Monoisotopic Molecliar Weight |
289.152537473
| IUPAC Name |
5-{[1-carboxy-3-(trimethylazaniumyl)propan-2-yl]oxy}-3-methyl-5-oxopentanoate
| Traditional Name |
5-{[1-carboxy-3-(trimethylammonio)propan-2-yl]oxy}-3-methyl-5-oxopentanoate
| 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 |
This compound belongs to the class of chemical entities known as acyl carnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Lipids and lipid-like moleclies
| Sub Class |
Fatty Acyls
| Direct Parent |
Acyl carnitines
| Alternative Parents |
Tricarboxylic acids and derivatives
Tetraalkylammonium salts
Carboxylic acid salts
Carboxylic acid esters
Carboxylic acids
Organopnictogen compounds
Organic salts
Organic oxides
Hydrocarbon derivatives
Carbonyl compounds
Amines
| 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
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
O-methylglutarylcarnitine (CHEBI:70857 )
| Ontology |
| Status |
Detected and Quantified
| Origin |
Endogenous
Food
| Biofunction |
Cell signaling
Fuel and energy storage
Fuel or energy source
Lipid catabolism, Fatty acid transport, Energy production
Membrane integrity/stability
| Application |
Nutrients
Stabilizers
Surfactants and Emlisifiers
| Cellliar locations |
Cytoplasm
Extracellliar
Membrane
| Physical Properties |
| State |
Solid
| Experimental Properties |
| Property |
Value |
Reference |
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
| Predicted Properties |
| Property |
Value |
Source |
Water Solubility0.066 mg/mLALOGPS
logP-1.6ALOGPS
logP-3.9ChemAxon
logS-3.7ALOGPS
pKa (Strongest Acidic)3.42ChemAxon
pKa (Strongest Basic)-7.1ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count5ChemAxon
Hydrogen Donor Count1ChemAxon
Polar Surface Area103.73 Å2ChemAxon
Rotatable Bond Count10ChemAxon
Refractivity92.68 m3·mol-1ChemAxon
Polarizability29.62 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
Not Available
| Biological Properties |
| Cellliar Locations |
Cytoplasm
Extracellliar
Membrane
| Biofluid Locations |
Blood
Saliva
Urine
| Tissue Location |
Not Available
| Pathways |
Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
BloodExpected but not Quantified Not AvailableNot Available
Normal
details
SalivaDetected and Quantified0.088 +/- 0.011 uMAdlit (>18 years old)BothNormal
Zerihun T. Dame, …
details
UrineDetected and Quantified0.030 (0.016-0.052) umol/mmol creatinineAdlit (>18 years old)Both
Normal
24023812
details
|
| Abnormal Concentrations |
|
Not Available
| Associated Disorders and Diseases |
| Disease References |
None
| Associated OMIM IDs |
None
| External Links |
| DrugBank ID |
Not Available
| DrugBank Metabolite ID |
Not Available
| Phenol Explorer Compound ID |
Not Available
| Phenol Explorer Metabolite ID |
Not Available
| FoodDB 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
| NuGOwiki Link |
HMDB00552
| Metagene Link |
HMDB00552
| METLIN ID |
5536
| PubChem Compound |
128145
| PDB ID |
Not Available
| ChEBI ID |
Not Available
Product: Mogrol
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 |
- 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 ]
|
PMID: 11527975