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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 | 2019-01-11 19:14:34 UTC |
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HMDB ID | HMDB0000459 |
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Secondary Accession Numbers | |
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Metabolite Identification |
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Common Name | 3-Methylcrotonylglycine |
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Description | 3-Methylcrotonylglycine is an acyl glycine. Acyl glycines are normally minor metabolites of fatty acids. However, the excretion of certain acyl glycines is increased in several inborn errors of metabolism. In certain cases the measurement of these metabolites in body fluids can be used to diagnose disorders associated with mitochondrial fatty acid beta-oxidation. Acyl glycines are produced through the action of glycine N-acyltransferase (EC 2.3.1.13) which is an enzyme that catalyzes the chemical reaction: acyl-CoA + glycine < -- > CoA + N-acylglycine. 3-Methylcrotonylglycine is a normal amino acid metabolite found in urine. Increased levels of this metabolite are found in patients suffering from leucine catabolic disorders, such as 3-methylcrotonyl-CoA carboxylase deficiency. 3-Methylcrotonylglycine is often considered to be a diagnostic marker of organic acidemias (PMID 11170888 ). Moreover, 3-methylcrotonylglycine is found to be associated with 3-hydroxy-3-methylglutaryl-CoA lyase deficiency and propionic acidemia, which are also inborn errors of metabolism. |
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Structure | |
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Synonyms | Value | Source |
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beta-Methylcrotonylglycine | ChEBI | b-Methylcrotonylglycine | Generator | β-methylcrotonylglycine | Generator | 3-Methylcrotonyl glycine | HMDB | 3-Methylcrotonyl-glycine | HMDB | 3-Methylcrotonylglycin | HMDB | N-(3-Methyl-1-oxo-2-butenyl)-glycine | HMDB |
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Chemical Formula | C7H11NO3 |
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Average Molecular Weight | 157.1671 |
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Monoisotopic Molecular Weight | 157.073893223 |
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IUPAC Name | 2-(3-methylbut-2-enamido)acetic acid |
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Traditional Name | (3-methylbut-2-enamido)acetic acid |
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CAS Registry Number | 33008-07-0 |
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SMILES | CC(C)=CC(=O)NCC(O)=O |
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InChI Identifier | InChI=1S/C7H11NO3/c1-5(2)3-6(9)8-4-7(10)11/h3H,4H2,1-2H3,(H,8,9)(H,10,11) |
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InChI Key | PFWQSHXPNKRLIV-UHFFFAOYSA-N |
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Chemical Taxonomy |
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Description | This compound belongs to the class of organic compounds known as n-acyl-alpha amino acids. These are compounds containing an alpha amino acid which bears an acyl group at its terminal nitrogen atom. |
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Kingdom | Organic compounds |
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Super Class | Organic acids and derivatives |
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Class | Carboxylic acids and derivatives |
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Sub Class | Amino acids, peptides, and analogues |
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Direct Parent | N-acyl-alpha amino acids |
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Alternative Parents | |
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Substituents | - N-acyl-alpha-amino acid
- N-acyl-amine
- Secondary carboxylic acid amide
- Carboxamide group
- Monocarboxylic acid or derivatives
- Carboxylic acid
- Organic nitrogen compound
- Organic oxygen compound
- Organopnictogen compound
- Organic oxide
- Hydrocarbon derivative
- Organooxygen compound
- Organonitrogen compound
- Carbonyl group
- Aliphatic acyclic compound
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Molecular Framework | Aliphatic acyclic compounds |
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External Descriptors | |
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Ontology |
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Disposition | Source: Biological location: |
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Role | 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 | Spectrum Type | Description | Splash Key | |
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Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (Non-derivatized) - 70eV, Positive | splash10-001l-9200000000-b5de5c506d0864c0f7d1 | View in MoNA |
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Predicted GC-MS | Predicted GC-MS Spectrum - GC-MS (1 TMS) - 70eV, Positive | splash10-01x3-9210000000-3cd3c3c8ea08c7d1e6c7 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated) | splash10-001i-9000000000-de6b64abad08a6b0aa93 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated) | splash10-0a59-9000000000-6614867cbb98a3422626 | View in MoNA |
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LC-MS/MS | LC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated) | splash10-0a59-9000000000-87fe2c98c5568e0a3651 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Positive | splash10-0a4i-7900000000-f113c2d59fe1238ea573 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Positive | splash10-0a6r-9100000000-ca7eb3d4b533d2e31892 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Positive | splash10-0a4i-9000000000-78c1b06293f14b96b012 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 10V, Negative | splash10-0a4i-0900000000-9462ca1f1e0bcf30b871 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 20V, Negative | splash10-0a4i-6900000000-7cf2a248f3b7490e7677 | View in MoNA |
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Predicted LC-MS/MS | Predicted LC-MS/MS Spectrum - 40V, Negative | splash10-0ab9-9000000000-30da48e2c1ce30e69097 | View in MoNA |
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1D NMR | 1H NMR Spectrum | Not Available | View in JSpectraViewer |
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2D NMR | [1H,13C] 2D NMR Spectrum | Not Available | View in JSpectraViewer |
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Biological Properties |
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Cellular Locations | |
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Biospecimen Locations | |
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Tissue Locations | |
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Pathways | |
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Normal Concentrations |
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Blood | Expected but not Quantified | | Not Available | Not Available | Normal | | details | Urine | Detected and Quantified | 0.0559–0.6455 umol/mmol creatinine | Adult (25-30 years old) | Both | Not Available | | details | Urine | Detected and Quantified | 0.040.0–0.6236 umol/mmol creatinine | Adult (25-30 years old) | Both | Not Available | | details | Urine | Detected and Quantified | 0.0651–0.4442 umol/mmol creatinine | Adult (25-30 years old) | Both | Not Available | | details | Urine | Detected but not Quantified | | Adult (24-38years old) | Not Specified | Normal | | details | Urine | Detected and Quantified | <0.48 umol/mmol creatinine | Adult (>18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 0.9-2.0 umol/mmol creatinine | Adult (>18 years old) | Female | Normal | | details | Urine | Detected and Quantified | <2 umol/mmol creatinine | Not Specified | Not Specified | Normal | | details | Urine | Detected and Quantified | <10 umol/mmol creatinine | Children (1 - 18 years old) | Both | Normal | | details | Urine | Detected and Quantified | 6.100 +/- 4.100 umol/mmol creatinine | Not Specified | Not Specified | Normal | | details | Urine | Detected and Quantified | 0.8-1.2 umol/mmol creatinine | Adult (>18 years old) | Male | Normal | | details |
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Abnormal Concentrations |
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Urine | Detected and Quantified | 700.0 (400.0-1000.0) umol/mmol creatinine | Children (1-13 years old) | Both | 3-Methyl-Crotonyl-Glycinuria | | details | Urine | Detected and Quantified | 27.5 (5.0-50.0) umol/mmol creatinine | Children (1-13 years old) | Both | Biotinidase deficiency | | details | Urine | Detected and Quantified | 0.41 umol/mmol creatinine | Adult (>18 years old) | Not Specified | Propionic acidemia | | details | Urine | Detected and Quantified | 27.6 umol/mmol creatinine | Newborn (0-30 days old) | Male | 3-hydroxy-3-methylglutaric- coenzyme A lyase deficiency | | details | Urine | Detected and Quantified | 120 umol/mmol creatinine | Children (1 - 13 years old) | Female | 3-Methylcrotonyl-CoA Carboxylase Deficiency | | details | Urine | Detected and Quantified | 1975 umol/mmol creatinine | Children (1 - 13 years old) | Not Specified | Isolated 3-Methylcrotonyl-CoA Carboxylase Deficiency | | details | Urine | Detected and Quantified | 27.6 umol/mmol creatinine | Newborn (0-30 days old) | Male | 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency | | details | Urine | Detected and Quantified | 225.0 (0.00-450.0) umol/mmol creatinine | Children (1-13 years old) | Both | 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency | | 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 ]
- G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
| 3-Methyl-crotonyl-glycinuria |
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- Rutledge SL, Berry GT, Stanley CA, van Hove JL, Millington D: Glycine and L-carnitine therapy in 3-methylcrotonyl-CoA carboxylase deficiency. J Inherit Metab Dis. 1995;18(3):299-305. [PubMed:7474896 ]
- de Kremer RD, Latini A, Suormala T, Baumgartner ER, Larovere L, Civallero G, Guelbert N, Paschini-Capra A, Depetris-Boldini C, Mayor CQ: Leukodystrophy and CSF purine abnormalities associated with isolated 3-methylcrotonyl-CoA carboxylase deficiency. Metab Brain Dis. 2002 Mar;17(1):13-8. [PubMed:11893004 ]
- G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
| Biotinidase deficiency |
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- G.Frauendienst-Egger, Friedrich K. Trefz (2017). MetaGene: Metabolic & Genetic Information Center (MIC: http://www.metagene.de). METAGENE consortium.
| Propionic acidemia |
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- Riemersma M, Hazebroek MR, Helderman-van den Enden ATJM, Salomons GS, Ferdinandusse S, Brouwers MCGJ, van der Ploeg L, Heymans S, Glatz JFC, van den Wijngaard A, Krapels IPC, Bierau J, Brunner HG: Propionic acidemia as a cause of adult-onset dilated cardiomyopathy. Eur J Hum Genet. 2017 Nov;25(11):1195-1201. doi: 10.1038/ejhg.2017.127. Epub 2017 Aug 30. [PubMed:28853722 ]
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Associated OMIM IDs | - 246450 (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
- 210200 (3-Methyl-crotonyl-glycinuria)
- 253260 (Biotinidase deficiency)
- 606054 (Propionic acidemia)
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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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FoodDB ID | FDB022057 |
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KNApSAcK ID | Not Available |
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Chemspider ID | 148224 |
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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 | 5447 |
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PubChem Compound | 169485 |
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PDB ID | Not Available |
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ChEBI ID | 68499 |
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References |
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Synthesis Reference | Carter, S. M. Bonham; Watson, D. G.; Midgley, J. M.; Logan, R. W. Synthesis and characterization of acyl glycines. Their measurement in single blood spots by gas chromatography-mass spectrometry to diagnose inborn errors of metabolism. Journal of Chromatography, B: Biomedical Applications (1996), 677(1), 29-35. |
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Material Safety Data Sheet (MSDS) | Not Available |
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General References | - Gallardo ME, Desviat LR, Rodriguez JM, Esparza-Gordillo J, Perez-Cerda C, Perez B, Rodriguez-Pombo P, Criado O, Sanz R, Morton DH, Gibson KM, Le TP, Ribes A, de Cordoba SR, Ugarte M, Penalva MA: The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am J Hum Genet. 2001 Feb;68(2):334-46. Epub 2001 Jan 17. [PubMed:11170888 ]
- Chantin C, Bonin B, Boulieu R, Bory C: Liquid-chromatographic study of purine metabolism abnormalities in purine nucleoside phosphorylase deficiency. Clin Chem. 1996 Feb;42(2):326-8. [PubMed:8595732 ]
- Wysocki SJ, Hahnel R: 3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria. Clin Chim Acta. 1978 May 16;86(1):101-8. [PubMed:657530 ]
- Koeberl DD, Millington DS, Smith WE, Weavil SD, Muenzer J, McCandless SE, Kishnani PS, McDonald MT, Chaing S, Boney A, Moore E, Frazier DM: Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening. J Inherit Metab Dis. 2003;26(1):25-35. [PubMed:12872837 ]
- Baykal T, Gokcay GH, Ince Z, Dantas MF, Fowler B, Baumgartner MR, Demir F, Can G, Demirkol M: Consanguineous 3-methylcrotonyl-CoA carboxylase deficiency: early-onset necrotizing encephalopathy with lethal outcome. J Inherit Metab Dis. 2005;28(2):229-33. [PubMed:15877210 ]
- Cowan MJ, Wara DW, Packman S, Ammann AJ, Yoshino M, Sweetman L, Nyhan W: Multiple biotin-dependent carboxylase deficiencies associated with defects in T-cell and B-cell immunity. Lancet. 1979 Jul 21;2(8134):115-8. [PubMed:88554 ]
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