| Common Name |
3-Methylglutaconic acid
| Description |
3-Methylglutaconic acid is an intermediate (as the CoA thioester) in the leucine degradative pathway as well as the mevalonate shunt, a pathway that links isoprenoid metabolism with mitochondrial acetyl-CoA metabolism. (PMID: 7603789 ). 3-methylglutaconyl-CoA hydratase is involved in the metabolism process of 3-methylglutaconic acid. 3-Methylglutaconic acid accumliates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine. (Wikipedia).
| Structure |
MOLSDF3D-SDFPDBSMILESInChI View 3D Structure
| Synonyms |
| Value |
Source |
3-MethylglutaconateHMDB
b-MethylglutaconateHMDB
b-Methylglutaconic acidHMDB
beta-MethylglutaconateHMDB
beta-Methylglutaconic acidHMDB
| Chemical Formlia |
C6H8O4
| Average Molecliar Weight |
144.1253
| Monoisotopic Molecliar Weight |
144.042258744
| IUPAC Name |
(2E)-3-methylpent-2-enedioic acid
| Traditional Name |
β-methylglutaconic acid
| CAS Registry Number |
5746-90-7
| SMILES |
CC(CC(O)=O)=C/C(O)=O
| InChI Identifier |
InChI=1S/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
| InChI Key |
WKRBKYFIJPGYQC-DUXPYHPUSA-N
| Chemical Taxonomy |
| Description |
This compound belongs to the class of chemical entities known as methyl-branched fatty acids. These are fatty acids with an acyl chain that has a methyl branch. Usually, they are saturated and contain only one or more methyl group. However, branches other than methyl may be present.
| Kingdom |
Chemical entities
| Super Class |
Organic compounds
| Class |
Lipids and lipid-like moleclies
| Sub Class |
Fatty Acyls
| Direct Parent |
Methyl-branched fatty acids
| Alternative Parents |
Unsaturated fatty acids
Dicarboxylic acids and derivatives
Carboxylic acids
Organic oxides
Hydrocarbon derivatives
Carbonyl compounds
| Substituents |
Methyl-branched fatty acid
Unsaturated fatty acid
Dicarboxylic acid or derivatives
Carboxylic acid
Carboxylic acid derivative
Organic oxygen compound
Organic oxide
Hydrocarbon derivative
Organooxygen compound
Carbonyl group
Aliphatic acyclic compound
| Molecliar Framework |
Aliphatic acyclic compounds
| External Descriptors |
dicarboxylic acid (CHEBI:37245 )
Dicarboxylic acids (LMFA01170068 )
| Ontology |
| Status |
Detected and Quantified
| Origin |
Endogenous
Food
| Biofunction |
Cell signaling
Fuel and energy storage
Fuel or energy source
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 Solubility8.69 mg/mLALOGPS
logP0.37ALOGPS
logP0.29ChemAxon
logS-1.2ALOGPS
pKa (Strongest Acidic)3.85ChemAxon
Physiological Charge-2ChemAxon
Hydrogen Acceptor Count4ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area74.6 Å2ChemAxon
Rotatable Bond Count3ChemAxon
Refractivity33.51 m3·mol-1ChemAxon
Polarizability13.27 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rlie of FiveYesChemAxon
Ghose FilterYesChemAxon
Vebers RlieYesChemAxon
MDDR-like RlieYesChemAxon
| Spectra |
| Spectra |
| Spectrum Type |
Description |
Splash Key |
|
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, PositiveNot Available
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, PositiveNot Available
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, PositiveNot Available
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 10V, NegativeNot Available
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 20V, NegativeNot Available
| Predicted LC-MS/MS |
Predicted LC-MS/MS Spectrum – 40V, NegativeNot Available
| Biological Properties |
| Cellliar Locations |
Cytoplasm
Extracellliar
Membrane
| Biofluid Locations |
Urine
| Tissue Location |
Not Available
| Pathways |
Not Available
| Normal Concentrations |
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
UrineDetected but not Quantified Adlit (>18 years old)Both
Normal
24023812
details
UrineDetected and Quantified<12.42 umol/mmol creatinineChildren (1 – 18 years old)Both
Normal
BC Children's Hos…
details
UrineDetected and Quantified6.2 (2.8-8.3) umol/mmol creatinineAdlit (>18 years old)Both
Normal
24023812
details
UrineDetected and Quantified10.707 +/- 4.243 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Normal
Mordechai, Hien, …
details
UrineDetected and Quantified7.5 (3.4-9.5) umol/mmol creatinineAdlit (>18 years old)Both
Normal
24023812
details
UrineDetected and Quantified15.86 +/- 6.052 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Normal
Mordechai, Hien, …
details
|
| Abnormal Concentrations |
|
| Biofluid |
Status |
Age |
Condition |
Reference |
Details |
UrineDetected and Quantified750.0 (500.0-1000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Methylglutaconic Aciduria (TYPE I)
MetaGene
details
UrineDetected and Quantified10.714 +/- 4.929 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Eosinophilic esophagitis
Mordechai, Hien, …
details
UrineDetected and Quantified12.053 +/- 6.469 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Gastroesophageal reflux disease
Mordechai, Hien, …
details
UrineDetected and Quantified14.626 +/- 7.998 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Eosinophilic esophagitis
Mordechai, Hien, …
details
UrineDetected and Quantified4.5 (0.00-9.0) umol/mmol creatinineAdlit (>18 years old)Both3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
MetaGene
details
UrineDetected and Quantified17.038 +/- 8.547 umol/mmol creatinineChildren (1 – 13 years old)Not Specified
Gastroesophageal reflux disease
Mordechai, Hien, …
details
UrineDetected and Quantified5070.0 (140.0-10000.0) umol/mmol creatinineChildren (1-13 years old)Both3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency
MetaGene
details
| Associated Disorders and Diseases |
| Disease References |
| 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency |
- MetaGene [Link]
| 3-Methylglutaconic aciduria type I |
- MetaGene [Link]
| Associated OMIM IDs |
246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
250950 (3-Methylglutaconic aciduria type I)
| 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 |
FDB022090
| KNApSAcK ID |
Not Available
| Chemspider ID |
1267861
| KEGG Compound ID |
Not Available
| BioCyc ID |
Not Available
| BiGG ID |
Not Available
| Wikipedia Link |
3-Methylglutaconic acid
| NuGOwiki Link |
HMDB00522
| Metagene Link |
HMDB00522
| METLIN ID |
5507
| PubChem Compound |
1551553
| PDB ID |
Not Available
| ChEBI ID |
37245
Product: Pemafibrate (racemate)
References |
| Synthesis Reference |
Aoyama, Takayuki; Kato, Kazuo; Shimada, Nobuyoshi; Fujii, Akio; Takita, Tomohisa. Production of 3-methylglutaconic acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp. |
| Material Safety Data Sheet (MSDS) |
Not Available |
| General References |
- Mitchell GA, Jakobs C, Gibson KM, Robert MF, Burlina A, Dionisi-Vici C, Dallaire L: Molecular prenatal diagnosis of 3-hydroxy-3-methylglutaryl CoA lyase deficiency. Prenat Diagn. 1995 Aug;15(8):725-9. [PubMed:7479590 ]
- Holme E, Greter J, Jacobson CE, Larsson NG, Lindstedt S, Nilsson KO, Oldfors A, Tulinius M: Mitochondrial ATP-synthase deficiency in a child with 3-methylglutaconic aciduria. Pediatr Res. 1992 Dec;32(6):731-5. [PubMed:1287564 ]
- Costeff H, Elpeleg O, Apter N, Divry P, Gadoth N: 3-Methylglutaconic aciduria in “optic atrophy plus”. Ann Neurol. 1993 Jan;33(1):103-4. [PubMed:8494328 ]
- Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behrs syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. [PubMed:1384336 ]
- Kelley RI: Quantification of 3-methylglutaconic acid in urine, plasma, and amniotic fluid by isotope-dilution gas chromatography/mass spectrometry. Clin Chim Acta. 1993 Nov 15;220(2):157-64. [PubMed:8111960 ]
- Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed:7603789 ]
|
PMID: 2181489