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Record Information |
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Version | 4.0 |
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Status | Detected and Quantified |
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Creation Date | 2005-11-16 15:48:42 UTC |
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Update Date | 2020-11-09 23:13:46 UTC |
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HMDB ID | HMDB0000552 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | 3-Methylglutarylcarnitine |
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Description | 3-Methylglutarylcarnitine belongs to the class of organic compounds known as acylcarnitines. These are organic compounds containing a fatty acid with the carboxylic acid attached to carnitine through an ester bond 3-Methylglutarylcarnitine is an extremely weak basic (essentially neutral) compound (based on its pKa). 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 ). |
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Structure | |
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Synonyms | Value | Source |
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(3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoic acid | Generator | 3-Methylglutarylcarnitine | HMDB | Methylglutarylcarnitine | HMDB | O-3-Methylglutarylcarnitine | HMDB |
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Chemical Formula | C13H23NO6 |
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Average Molecular Weight | 289.328 |
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Monoisotopic Molecular Weight | 289.152537465 |
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IUPAC Name | (3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylazaniumyl)butanoate |
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Traditional Name | (3R)-3-{[(3R)-4-carboxy-3-methylbutanoyl]oxy}-4-(trimethylammonio)butanoate |
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CAS Registry Number | 102673-95-0 |
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SMILES | C[C@H](CC(O)=O)CC(=O)O[C@H](CC([O-])=O)C[N+](C)(C)C |
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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)/t9-,10-/m1/s1 |
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InChI Key | HFCPFJNSBPQJDP-NXEZZACHSA-N |
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Chemical Taxonomy |
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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. |
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Kingdom | Organic compounds |
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Super Class | Lipids and lipid-like molecules |
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Class | Fatty Acyls |
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Sub Class | Fatty acid esters |
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Direct Parent | Acyl carnitines |
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Alternative Parents | |
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Substituents | - Acyl-carnitine
- Tricarboxylic acid or derivatives
- Tetraalkylammonium salt
- Quaternary ammonium salt
- Carboxylic acid salt
- Carboxylic acid ester
- Carboxylic acid
- Carboxylic acid derivative
- Organic nitrogen compound
- Organic oxygen compound
- Organopnictogen compound
- Organic oxide
- Hydrocarbon derivative
- Organic salt
- Organooxygen compound
- Organonitrogen compound
- Carbonyl group
- Amine
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | Not Available |
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Ontology |
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Disposition | Route of exposure: Source: Biological location: |
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Process | Naturally occurring process: |
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Role | Industrial application: Biological role: |
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Physical Properties |
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State | Solid |
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Experimental Properties | Property | Value | Reference |
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Melting Point | Not Available | Not Available | Boiling Point | Not Available | Not Available | Water Solubility | Not Available | Not Available | LogP | Not Available | Not Available |
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Predicted Properties | |
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Spectra |
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| Not Available |
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Biological Properties |
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Cellular Locations | - Cytoplasm
- Extracellular
- Membrane
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Biospecimen Locations | |
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Tissue Locations | Not Available |
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Pathways | |
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Normal Concentrations |
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Blood | Detected and Quantified | 0.0000-0.140 uM | Not Specified | Not Specified | Normal | | details | Blood | Expected but not Quantified | Not Quantified | Not Available | Not Available | Normal | | details | Blood | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Blood | Detected and Quantified | 0.020-0.060 uM | Adult (>18 years old) | Both | Normal | | details | Feces | Detected and Quantified | 0.24 +/- 0.1 nmol/g wet feces | Adult (>18 years old) | Both | Normal | | details | Feces | Detected and Quantified | 1.1 +/- 0.65 nmol/g wet feces | Adult (>18 years old) | Both | Normal | | details | Saliva | Detected and Quantified | 0.088 +/- 0.011 uM | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0.03-0.13 umol/mmol creatinine | Newborn (0-30 days old) | Both | Normal | | details | Urine | Detected and Quantified | 0.05(0.03-0.13) umol/mmol creatinine | Newborn (0-30 days old) | Female | Normal | | details | Urine | Detected and Quantified | 0.06(0.03-0.13) umol/mmol creatinine | Newborn (0-30 days old) | Male | Normal | | details | Urine | Detected but not Quantified | Not Quantified | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0.030 (0.016-0.052) umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details |
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Abnormal Concentrations |
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Blood | Detected and Quantified | 0.76 uM | Newborn (0-30 days old) | Male | 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency | | details | Blood | Detected and Quantified | 0.049 (0.0327) uM | Adult (>18 years old) | Female | Pregnancy with fetus having congenital heart defect | | details |
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Associated Disorders and Diseases |
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Disease References | 3-Hydroxy-3-methylglutaryl-CoA lyase deficiency |
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- Santarelli F, Cassanello M, Enea A, Poma F, D'Onofrio V, Guala G, Garrone G, Puccinelli P, Caruso U, Porta F, Spada M: A neonatal case of 3-hydroxy-3-methylglutaric-coenzyme A lyase deficiency. Ital J Pediatr. 2013 May 24;39:33. doi: 10.1186/1824-7288-39-33. [PubMed:23705938 ]
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Associated OMIM IDs | - 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
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External Links |
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DrugBank ID | Not Available |
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Phenol Explorer Compound ID | Not Available |
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FooDB ID | FDB022112 |
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KNApSAcK ID | Not Available |
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Chemspider ID | Not Available |
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KEGG Compound ID | Not Available |
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BioCyc ID | Not Available |
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BiGG ID | Not Available |
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Wikipedia Link | Not Available |
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METLIN ID | Not Available |
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PubChem Compound | Not Available |
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PDB ID | Not Available |
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ChEBI ID | Not Available |
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Food Biomarker Ontology | Not Available |
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VMH ID | Not Available |
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MarkerDB ID | MDB00029840 |
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References |
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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. |
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Material Safety Data Sheet (MSDS) | Not Available |
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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 ]
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