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Human Metabolome Database Version 2.5

Showing metabocard for Methylglutaric acid (HMDB00752)

Version	2.5
Creation Date	2005-11-16 15:48:42
Update Date	2010-01-20 15:15:21
Accession Number	HMDB00752
Secondary Accession Numbers	Not Available
Common Name	Methylglutaric acid
Description	Methylglutaric acid is a leucine metabolite. A large amount of methylglutaric acid is identified in urine of patients with deficiency of 3-methylglutaconyl coenzyme A hydratase (PMID 6181239).
Synonyms	3-Methylglutarate 3-Methylglutaric acid 3-Methylpentanedioate 3-Methylpentanedioic acid 3-methyl-Glutarate 3-methyl-Glutaric acid Methylglutarate b-Methylglutarate b-Methylglutaric acid b-methyl-Glutaric acid beta-Methylglutarate beta-Methylglutaric acid beta-methyl-Glutaric acid
Chemical IUPAC Name	3-methyl-Pentanedioic acid
Chemical Formula	C6H10O4
Chemical Structure
Chemical Taxonomy	Kingdom Organic Super Class Organic acids Class Dicarboxylic Acids Sub Class Short chain dicarboxylic acids Family Mammalian Metabolite Species carboxylic acid Biofunction — Application — Source Endogenous
Average Molecular Weight	146.141
Monoisotopic Molecular Weight	146.057907
Isomeric SMILES	CC(CC(O)=O)CC(O)=O
Canonical SMILES	CC(CC(O)=O)CC(O)=O
KEGG Compound ID	Not Available
BioCyc ID	CPD-516
BiGG ID	Not Available
Wikipedia Link	Not Available
NuGOwiki Link	HMDB00752
Metagene Link	HMDB00752
METLIN ID	5720
PubChem Compound	12284
PubChem Substance	10530728
ChEBI ID	Not Available
CAS Registry Number	626-51-7
InChI Identifier	InChI=1/C6H10O4/c1-4(2-5(7)8)3-6(9)10/h4H,2-3H2,1H3,(H,7,8)(H,9,10)
Synthesis Reference	Komppa, Gust. b-Methylglutaric acid. Ann. acad. sci. Fennicae (1930), 30(Ser. A;No. 9), 5 pp.
Melting Point (Experimental)	80-82 oC
Experimental Water Solubility	Not Available Source: PhysProp
Predicted Water Solubility	214.0 mg/mL [MEYLAN,WM et al. (1996)]; 36.9 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge	-2
State	Solid
Experimental LogP/Hydrophobicity	Not Available Source: PhysProp
Predicted LogP/Hydrophobicity	0.33 [Predicted by ALOGPS]; 0.2 [Predicted by PubChem via XLOGP] Calculated using ALOGPS
Material Safety Data Sheet (MSDS)	Show PDF/HTML
MOL File	Show
SDF File	Show
PDB File	Show
2D Structure
3D Structure
Experimental PDB ID	Not Available
Experimental 1H NMR Spectrum	Download Spectrum Download FID (Varian) Show Experimental Conditions
Experimental 13C NMR Spectrum	Not Available
Experimental 13C HSQC Spectrum	Download Spectrum Download FID (Bruker) Show Experimental Conditions
Predicted 1H NMR Spectrum	Show Image Show Peaklist
Predicted 13C NMR Spectrum	Show Image Show Peaklist
Mass Spectrum	Low Energy Download File Show Experimental Conditions Medium Energy Download File Show Experimental Conditions High Energy Download File Show Experimental Conditions
Simplified TOCSY Spectrum	Not Available
BMRB Spectrum	Not Available
Cellular Location	Not Available
Biofluid Location	Urine
Tissue Location	Not Available
Concentrations (Normal)	Biofluid Urine Value 1.0 - 6.5 umol/mmol creatinine Age Adult:>18 yrs old Sex N/A Patient information Normal Comments Not Available References Engelke UF, Kremer B, Kluijtmans LA, van der Graaf M, Morava E, Loupatty FJ, Wanders RJ, Moskau D, Loss S, van den Bergh E, Wevers RA: NMR spectroscopic studies on the late onset form of 3-methylglutaconic aciduria type I and other defects in leucine metabolism. NMR Biomed. 2006 Apr;19(2):271-8. [PubMed ] Biofluid Urine Value 0.1 (0.1-9.7) umol/mmol creatinine Age Newborn:0-30 days old Sex Both Patient information Normal Comments Not Available References Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] Biofluid Urine Value 0.1 (0.1-0.1) umol/mmol creatinine Age Infant:0-1 yr old Sex Both Patient information Normal Comments Not Available References Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] Biofluid Urine Value 1.0 (0.1-2.8) umol/mmol creatinine Age Children:1-13 yrs old Sex Both Patient information Normal Comments Children aged 2-6 years References Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] Biofluid Urine Value 1.1 (0.1-2.0) umol/mmol creatinine Age Children:1-13 yrs old Sex Both Patient information Normal Comments Children aged 6-10 years References Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] Biofluid Urine Value 0.6 (0.1-2.1) umol/mmol creatinine Age Adolescent:13-18 yrs old Sex Both Patient information Normal Comments Children >10 years old References Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] Biofluid Urine Value 3.5 (0.00-7.0) umol/mmol creatinine Age Adult:>18 yrs old Sex Both Patient information Normal Comments Not Available References Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
Concentrations (Abnormal)	Biofluid Urine Value 1.6 (1.3-2.0) umol/mmol creatinine Age Adult:>18 yrs old Sex Both Condition Abnormal Comments Not Available References Geigy Scientific Tables, 8th Rev edition, pp. 130. Edited by C. Lentner, West Cadwell, N.J.: Medical education Div., Ciba-Geigy Corp. Basel, Switzerland c1981-1992. Biofluid Urine Value 507.0 (14.0-1000.0) umol/mmol creatinine Age Adult:>18 yrs old Sex Both Condition 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency Comments Not Available References Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY Biofluid Urine Value 7.5 (5.0-10.0) umol/mmol creatinine Age Adult:>18 yrs old Sex Both Comments Not Available References Metagene: 3-METHYLGLUTACONIC ACIDURIA (TYPE I)
Associated Disorders	Condition References 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency Metagene: 3-HYDROXY-3-METHYLGLUTARYL-CoA LYASE DEFICIENCY
OMIM ID	246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
Pathways	Not Available
General References	Guneral F, Bachmann C: Age-related reference values for urinary organic acids in a healthy Turkish pediatric population. Clin Chem. 1994 Jun;40(6):862-6. [PubMed ] 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 ] Truscott RJ, Halpern B, Wysocki SJ, Hahnel R, Wilcken B: Studies on a child suspected of having a dficiency in 3-hydroxy-3-methylglutaryl-Co A lyase. Clin Chim Acta. 1979 Jul 2;95(1):11-16. [PubMed ] Duran M, Beemer FA, Tibosch AS, Bruinvis L, Ketting D, Wadman SK: Inherited 3-methylglutaconic aciduria in two brothers--another defect of leucine metabolism. J Pediatr. 1982 Oct;101(4):551-4. [PubMed ]

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