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Human Metabolome Database Version 2.5

 

Showing metabocard for 3-Methylcrotonylglycine (HMDB00459)

Legend: metabolite field enzyme field

Version 2.5
Creation Date 2005-11-16 15:48:42
Update Date 2009-06-23 15:43:38
Accession Number HMDB00459
Secondary Accession Numbers Not Available
Common Name 3-Methylcrotonylglycine
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)
Synonyms
  1. 3-Methylcrotonyl-glycine
  2. 3-Methylcrotonyl glycine
  3. 3-Methylcrotonylglycin
  4. 3-Methylcrotonylglycine
  5. N-(3-methyl-1-oxo-2-butenyl)-Glycine
  6. beta-Methylcrotonylglycine
Chemical IUPAC Name 2-(3-methylbut-2-enoylamino)acetic acid
Chemical Formula C7H11NO3
Chemical Structure Structure
Chemical Taxonomy
Kingdom
  • Organic
Super Class
  • Amino acids and Amino Acid conjugates
Class
  • Acyl Glycines
Sub Class
  • Aliphatic glycine conjugates
Family
  • Mammalian Metabolite
Species
  • carboxylic acid
  • secondary carboxylic acid amide
  • alkene
Biofunction
—
Application
—
Source
  • Endogenous
Average Molecular Weight 157.167
Monoisotopic Molecular Weight 157.073898
Isomeric SMILES CC(C)=C/C(=O)NCC(O)=O
Canonical SMILES CC(C)=CC(=O)NCC(O)=O
KEGG Compound ID Not Available
BioCyc ID Not Available
BiGG ID Not Available
Wikipedia Link Not Available
NuGOwiki Link HMDB00459 Link Image
Metagene Link HMDB00459 Link Image
METLIN ID 5447 Link Image
PubChem Compound 169485 Link Image
PubChem Substance 747005 Link Image
ChEBI ID Not Available
CAS Registry Number 33008-07-0
InChI Identifier InChI=1/C7H11NO3/c1-5(2)3-6(9)8-4-7(10)11/h3H,4H2,1-2H3,(H,8,9)(H,10,11)
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.
Melting Point (Experimental) Not Available
Experimental Water Solubility Not Available Source: PhysProp
Predicted Water Solubility 7.64 mg/mL [Predicted by ALOGPS] Calculated using ALOGPS
Physiological Charge -1
State Solid
Experimental LogP/Hydrophobicity Not Available Source: PhysProp
Predicted LogP/Hydrophobicity 0.02 [Predicted by ALOGPS]; -2.2 [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 Download Spectrum
Download FID (Varian)
Show Experimental Conditions Link Image
Experimental 13C NMR Spectrum Not Available
Experimental 13C HSQC Spectrum Download Spectrum
Download FID (Bruker)
Show Experimental Conditions Link Image
Predicted 1H NMR Spectrum Show Image
Show Peaklist
Predicted 13C NMR Spectrum Show Image
Show Peaklist
Mass Spectrum
Low Energy
Download File
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Medium Energy
Download File
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High Energy
Download File
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Simplified TOCSY Spectrum Not Available
BMRB Spectrum Not Available
Cellular Location
  • Cytoplasm
Biofluid Location
  • Urine
Tissue Location
Tissue References
Fibroblasts —
Concentrations (Normal)
Biofluid Urine
Value 1.0 (0.00-2.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
Biofluid Urine
Value 1.0 (0.0-2.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
Biofluid Urine
Value 1.0 (0.0-2.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Patient information Normal
Comments Not Available
References
Concentrations (Abnormal)
Biofluid Urine
Value 225.0 (0.00-450.0) 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 700.0 (400.0-1000.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Comments Not Available
References
Biofluid Urine
Value 27.5 (5.0-50.0) umol/mmol creatinine
Age Adult:>18 yrs old
Sex Both
Condition Biotinidase deficiency
Comments Not Available
References
Associated Disorders
Condition References
3-Hydroxy-3-methylglutaryl-CoA lyase deficiency
Biotinidase deficiency
OMIM ID
  • 246450 Link Image (3-Hydroxy-3-methylglutaryl-CoA lyase deficiency)
  • 253260 Link Image (Biotinidase deficiency)
Pathways Not Available
General References
  1. Wysocki SJ, Hahnel R: 3-Methylcrotonylglycine excretion in 3-hydroxy-3-methylglutaric aciduria. Clin Chim Acta. 1978 May 16;86(1):101-8. [PubMed Link Image]
  2. 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 Link Image]
  3. 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 Link Image]
  4. 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 Link Image]
  5. 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 Link Image]
  6. 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 Link Image]
Metabolic Enzymes
  1. Glycine N-acyltransferase
  2. Glycine N-acyltransferase-like protein 1
  3. Glycine N-acyltransferase-like protein 2
  4. Glycine N-acyltransferase-like protein 3
Enzyme 1 [top]
Enzyme 1 ID 12971
Enzyme 1 Name Glycine N-acyltransferase
Enzyme 1 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase
  2. AAc
  3. Aralkyl acyl-CoA N-acyltransferase
  4. Aralkyl acyl-CoA:amino acid N-acyltransferase
  5. HRP-1(CLP)
Enzyme 1 Gene Name GLYAT
Enzyme 1 Protein Sequence >Glycine N-acyltransferase 
MMLPLQGAQMLQMLEKSLRKSLPASLKVYGTVFHINHGNPFNLKAVVDKWPDFNTVVVCP
QEQDMTDDLDHYTNTYQIYSKDPQNCQEFLGSPELINWKQHLQIQSSQPSLNEAIQNLAA
IKSFKVKQTQRILYMAAETAKELTPFLLKSKILSPSGGKPKAINQEMFKLSSMDVTHAHL
VNKFWHFGGNERSQRFIERCIQTFPTCCLLGPEGTPVCWDLMDQTGEMRMAGTLPEYRLH
GLVTYVIYSHAQKLGKLGFPVYSHVDYSNEAMQKMSYTLQHVPIPRSWNQWNCVPL
Enzyme 1 Number of Residues 296
Enzyme 1 Molecular Weight 33898
Enzyme 1 Theoretical pI 8.28
Enzyme 1 GO Classification Not Available
Enzyme 1 General Function Not Available
Enzyme 1 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 1 Pathways Not Available
Enzyme 1 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 1 Pfam Domain Function
Enzyme 1 Signals
  • None
Enzyme 1 Transmembrane Regions
  • None
Enzyme 1 Essentiality Not Available
Enzyme 1 GenBank ID Protein 2554941 Link Image
Enzyme 1 UniProtKB/Swiss-Prot ID Q6IB77 Link Image
Enzyme 1 UniProtKB/Swiss-Prot Entry Name GLYAT_HUMAN Link Image
Enzyme 1 PDB ID Not Available
Enzyme 1 Cellular Location Not Available
Enzyme 1 Gene Sequence Not Available
Enzyme 1 GenBank Gene ID AF023466 Link Image
Enzyme 1 GeneCard ID Q6IB77 Link Image
Enzyme 1 GenAtlas ID GLYAT Link Image
Enzyme 1 HGNC ID HGNC:13734 Link Image
Enzyme 1 Chromosome Location Not Available
Enzyme 1 Locus Not Available
Enzyme 1 SNPs SNPJam Report Link Image
Enzyme 1 General References Not Available
Enzyme 1 Metabolite References Not Available
Enzyme 2 [top]
Enzyme 2 ID 12972
Enzyme 2 Name Glycine N-acyltransferase-like protein 1
Enzyme 2 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase-like protein 1
Enzyme 2 Gene Name GLYATL1
Enzyme 2 Protein Sequence >Glycine N-acyltransferase-like protein 1 
MILLNNSHKLLALYKSLARSIPESLKVYGSVYHINHGNPFNMEVLVDSWPEYQMVIIRPQ
KQEMTDDMDSYTNVYRMFSKEPQKSEEVLKNCEIVNWKQRLQIQGLQESLGEGIRVATFS
KSVKVEHSRALLLVTEDILKLNASSKSKLGSWAETGHPDDEFESETPNFKYAQLDVSYSG
LVNDNWKRGKNERSLHYIKRCIEDLPAACMLGPEGVPVSWVTMDPSCEVGMAYSMEKYRR
TGNMARVMVRYMKYLRQKNIPFYISVLEENEDSRRFVGQFGFFEASCEWHQWTCYPQNLV
PF
Enzyme 2 Number of Residues 302
Enzyme 2 Molecular Weight 35102
Enzyme 2 Theoretical pI 6.86
Enzyme 2 GO Classification Not Available
Enzyme 2 General Function Not Available
Enzyme 2 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 2 Pathways Not Available
Enzyme 2 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 2 Pfam Domain Function
Enzyme 2 Signals
  • None
Enzyme 2 Transmembrane Regions
  • None
Enzyme 2 Essentiality Not Available
Enzyme 2 GenBank ID Protein 71384826 Link Image
Enzyme 2 UniProtKB/Swiss-Prot ID Q969I3 Link Image
Enzyme 2 UniProtKB/Swiss-Prot Entry Name GLYL1_HUMAN Link Image
Enzyme 2 PDB ID Not Available
Enzyme 2 Cellular Location Not Available
Enzyme 2 Gene Sequence Not Available
Enzyme 2 GenBank Gene ID DQ084381 Link Image
Enzyme 2 GeneCard ID Q969I3 Link Image
Enzyme 2 GenAtlas ID GLYATL1 Link Image
Enzyme 2 HGNC ID HGNC:30519 Link Image
Enzyme 2 Chromosome Location Not Available
Enzyme 2 Locus Not Available
Enzyme 2 SNPs SNPJam Report Link Image
Enzyme 2 General References Not Available
Enzyme 2 Metabolite References Not Available
Enzyme 3 [top]
Enzyme 3 ID 12973
Enzyme 3 Name Glycine N-acyltransferase-like protein 2
Enzyme 3 Synonyms
  1. Acyl-CoA:glycine N-acyltransferase-like protein 2
Enzyme 3 Gene Name GLYATL2
Enzyme 3 Protein Sequence >Glycine N-acyltransferase-like protein 2 
MLVLHNSQKLQILYKSLEKSIPESIKVYGAIFNIKDKNPFNMEVLVDAWPDYQIVITRPQ
KQEMKDDQDHYTNTYHIFTKAPDKLEEVLSYSNVISWEQTLQIQGCQEGLDEAIRKVATS
KSVQVDYMKTILFIPELPKKHKTSSNDKMELFEVDDDNKEGNFSNMFLDASHAGLVNEHW
AFGKNERSLKYIERCLQDFLGFGVLGPEGQLVSWIVMEQSCELRMGYTVPKYRHQGNMLQ
IGYHLEKYLSQKEIPFYFHVADNNEKSLQALNNLGFKICPCGWHQWKCTPKKYC
Enzyme 3 Number of Residues 294
Enzyme 3 Molecular Weight 34278
Enzyme 3 Theoretical pI 6.67
Enzyme 3 GO Classification Not Available
Enzyme 3 General Function Not Available
Enzyme 3 Specific Function Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Can conjugate a multitude of substrates to form a variety of N-acylglycines
Enzyme 3 Pathways Not Available
Enzyme 3 Reactions
  • Acyl-CoA + glycine = CoA + N-acylglycine
Enzyme 3 Pfam Domain Function
Enzyme 3 Signals
  • None
Enzyme 3 Transmembrane Regions
  • None
Enzyme 3 Essentiality Not Available
Enzyme 3 GenBank ID Protein 29243559 Link Image
Enzyme 3 UniProtKB/Swiss-Prot ID Q8WU03 Link Image
Enzyme 3 UniProtKB/Swiss-Prot Entry Name GLYL2_HUMAN Link Image
Enzyme 3 PDB ID Not Available
Enzyme 3 Cellular Location Not Available
Enzyme 3 Gene Sequence Not Available
Enzyme 3 GenBank Gene ID AF426250 Link Image
Enzyme 3 GeneCard ID Q8WU03 Link Image
Enzyme 3 GenAtlas ID GLYATL2 Link Image
Enzyme 3 HGNC ID HGNC:24178 Link Image
Enzyme 3 Chromosome Location Not Available
Enzyme 3 Locus Not Available
Enzyme 3 SNPs SNPJam Report Link Image
Enzyme 3 General References Not Available
Enzyme 3 Metabolite References Not Available
Enzyme 4 [top]
Enzyme 4 ID 12974
Enzyme 4 Name Glycine N-acyltransferase-like protein 3
Enzyme 4 Synonyms Not Available
Enzyme 4 Gene Name GLYATL3
Enzyme 4 Protein Sequence >Glycine N-acyltransferase-like protein 3 
MLVLNCSTKLLILEKMLKSCFPESLKVYGAVMNINRGNPFQKEVVLDSWPDFKAVITRRQ
REAETDNLDHYTNAYAVFYKDVRAYRQLLEECDVFNWDQVFQIKGLQSELYDVSKAVANS
KQLNIKLTSFKAVHFSPVSSLPDTSFLKGPSPRLTYLSVANADLLNRTWSRGGNEQCLRY
IANLISCFPSVCVRDEKGNPVSWSITDQFATMCHGYTLPEHRRKGYSRLVALTLARKLQS
RGFPSQGNVLDDNTASISLLKSLHAEFLPCRFHRLILTPATFSGLPHL
Enzyme 4 Number of Residues 288
Enzyme 4 Molecular Weight 32704
Enzyme 4 Theoretical pI Not Available
Enzyme 4 GO Classification Not Available
Enzyme 4 General Function Not Available
Enzyme 4 Specific Function Acyltransferase which transfers the acyl group to the N-terminus of glycine (By similarity).
Enzyme 4 Pathways Not Available
Enzyme 4 Reactions Not Available
Enzyme 4 Pfam Domain Function Not Available
Enzyme 4 Signals
  • None
Enzyme 4 Transmembrane Regions
  • None
Enzyme 4 Essentiality Not Available
Enzyme 4 GenBank ID Protein Not Available
Enzyme 4 UniProtKB/Swiss-Prot ID Q5SZD4 Link Image
Enzyme 4 UniProtKB/Swiss-Prot Entry Name GLYL3_HUMAN Link Image
Enzyme 4 PDB ID Not Available
Enzyme 4 Cellular Location Not Available
Enzyme 4 Gene Sequence Not Available
Enzyme 4 GenBank Gene ID Not Available
Enzyme 4 GeneCard ID Q5SZD4 Link Image
Enzyme 4 GenAtlas ID GLYATL3 Link Image
Enzyme 4 HGNC ID HGNC:21349 Link Image
Enzyme 4 Chromosome Location Not Available
Enzyme 4 Locus Not Available
Enzyme 4 SNPs SNPJam Report Link Image
Enzyme 4 General References Not Available
Enzyme 4 Metabolite References Not Available