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Human Metabolome Database Version 2.5

 

Showing metabocard for 3-Methylglutaconic acid (HMDB00522)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2009-06-18 16:06:06
Accession Number HMDB00522
Secondary Accession Numbers Not Available
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 accumulates in patients with a deficiency of this enzyme and a large amount of 3-methylglutaconic acid appear in urine. (Wikipedia)
Synonyms
  1. 3-Methylglutaconate
  2. 3-Methylglutaconic acid
  3. b-Methylglutaconate
  4. b-Methylglutaconic acid
  5. beta-Methylglutaconate
  6. beta-Methylglutaconic acid
Chemical IUPAC Name 3-methyl-2-pentenedioic acid
Chemical Formula C6H8O4
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Organic acids
Class
  • Dicarboxylic Acids
Sub Class
  • Short chain dicarboxylic acids
Family
  • Mammalian Metabolite
Species
  • carboxylic acid
  • alkene
Biofunction
—
Application
—
Source
  • Endogenous
Average Molecular Weight 144.125
Monoisotopic Molecular Weight 144.042252
Isomeric SMILES CC(CC(O)=O)=C/C(O)=O
Canonical SMILES CC(CC(O)=O)=CC(O)=O
KEGG Compound ID Not Available
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link 3-Methylglutaconic acid Link Image
NuGOwiki Link HMDB00522 Link Image
Metagene Link HMDB00522 Link Image
METLIN ID 5507 Link Image
PubChem Compound 99884 Link Image
PubChem Substance 4587309 Link Image
ChEBI ID Not Available
CAS Registry Number 5746-90-7
InChI Identifier InChI=1/C6H8O4/c1-4(2-5(7)8)3-6(9)10/h2H,3H2,1H3,(H,7,8)(H,9,10)/b4-2+
Synthesis Reference Aoyama, Takayuki; Kato, Kazuo; Shimada, Nobuyoshi; Fujii, Akio; Takita, Tomohisa. Production of 3-methylglutaconic acid. Jpn. Kokai Tokkyo Koho (1986), 3 pp.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 8.69 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -2
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 0.37 [Predicted by ALOGPS]; -0.2 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS) Not Available
MOL File Show
SDF File Show
PDB File Show
2D Structure
3D Structure
Experimental PDB ID Not Available
Experimental 1H NMR Spectrum Not Available
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Not Available
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
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Mass Spectrum Not Available
Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
Biofluid Location
  • Blood
  • Urine
Tissue Location Not Available
Concentrations (Normal)
Biofluid Urine
Value 4.5 (0.00-9.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
Concentrations (Abnormal)
Biofluid Blood
Value 1.3 (0.5-2.0) uM
Age Children:1-13 yrs old
Sex Both
Condition Smith-Lemli-Opitz syndrome
Comments Not Available
References
  • Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed Link Image]
  • 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 Link Image]
Biofluid Urine
Value 5070.0 (140.0-10000.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Comments Not Available
References
Biofluid Urine
Value 750.0 (500.0-1000.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Associated Disorders
Condition References
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Smith-Lemli-Opitz syndrome
  • Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed Link Image]
  • 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 Link Image]
OMIM ID
  • 246450 Link Image (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 270400 Link Image (Smith-Lemli-Opitz syndrome)
Pathways Not Available
General References
  1. 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 Link Image]
  2. 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 Link Image]
  3. 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 Link Image]
  4. Sheffer RN, Zlotogora J, Elpeleg ON, Raz J, Ben-Ezra D: Behr's syndrome and 3-methylglutaconic aciduria. Am J Ophthalmol. 1992 Oct 15;114(4):494-7. [PubMed Link Image]
  5. 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 Link Image]
  6. Kelley RI, Kratz L: 3-methylglutaconic acidemia in Smith-Lemli-Opitz syndrome. Pediatr Res. 1995 May;37(5):671-4. [PubMed Link Image]
  7. Wikipedia Link Image