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Record Information
Version3.6
Creation Date2005-11-16 15:48:42 UTC
Update Date2016-02-11 01:03:23 UTC
HMDB IDHMDB00808
Secondary Accession NumbersNone
Metabolite Identification
Common NameN-Butyrylglycine
DescriptionN-Butyrylglycine 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-acylglycineAmino acids composed of glycine substituted at the nitrogen rather than the usual carbon position, resulting in the loss of hydrogen bonding donors. Polymers of these compounds are called Peptoids.
Structure
Thumb
Synonyms
ValueSource
ButyrylglycineHMDB
N-(1-Oxobutyl)glycineHMDB
N-ButanoylglycineHMDB
N-Butyryl-glycineHMDB
Chemical FormulaC6H11NO3
Average Molecular Weight145.1564
Monoisotopic Molecular Weight145.073893223
IUPAC Name2-butanamidoacetic acid
Traditional Namebutyrylglycine
CAS Registry Number20208-73-5
SMILES
CCCC(=O)NCC(O)=O
InChI Identifier
InChI=1S/C6H11NO3/c1-2-3-5(8)7-4-6(9)10/h2-4H2,1H3,(H,7,8)(H,9,10)
InChI KeyInChIKey=WPSSBBPLVMTKRN-UHFFFAOYSA-N
Chemical Taxonomy
DescriptionThis compound belongs to the class of organic compounds known as n-acyl-aliphatic-alpha amino acids. These are alpha amino acids carrying a N-acylated aliphatic chain.
KingdomOrganic compounds
Super ClassOrganic acids and derivatives
ClassCarboxylic acids and derivatives
Sub ClassAmino acids, peptides, and analogues
Direct ParentN-acyl-aliphatic-alpha amino acids
Alternative Parents
Substituents
  • N-acyl-aliphatic-alpha amino acid
  • Fatty acyl
  • N-acyl-amine
  • Fatty amide
  • Secondary carboxylic acid amide
  • Carboxamide group
  • Monocarboxylic acid or derivatives
  • Carboxylic acid
  • Carboxylic acid amide
  • Hydrocarbon derivative
  • Organooxygen compound
  • Organonitrogen compound
  • Carbonyl group
  • Aliphatic acyclic compound
Molecular FrameworkAliphatic acyclic compounds
External DescriptorsNot Available
Ontology
StatusDetected and Quantified
Origin
  • Endogenous
BiofunctionNot Available
ApplicationNot Available
Cellular locations
  • Cytoplasm (predicted from logP)
Physical Properties
StateSolid
Experimental Properties
PropertyValueReference
Melting PointNot AvailableNot Available
Boiling PointNot AvailableNot Available
Water SolubilityNot AvailableNot Available
LogPNot AvailableNot Available
Predicted Properties
PropertyValueSource
Water Solubility17.6 mg/mLALOGPS
logP0.03ALOGPS
logP-0.18ChemAxon
logS-0.92ALOGPS
pKa (Strongest Acidic)4.06ChemAxon
pKa (Strongest Basic)-1.2ChemAxon
Physiological Charge-1ChemAxon
Hydrogen Acceptor Count3ChemAxon
Hydrogen Donor Count2ChemAxon
Polar Surface Area66.4 Å2ChemAxon
Rotatable Bond Count4ChemAxon
Refractivity34.68 m3·mol-1ChemAxon
Polarizability14.64 Å3ChemAxon
Number of Rings0ChemAxon
Bioavailability1ChemAxon
Rule of FiveYesChemAxon
Ghose FilterYesChemAxon
Veber's RuleYesChemAxon
MDDR-like RuleYesChemAxon
Spectra
Spectra
Spectrum TypeDescriptionSplash Key
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 10V, Positive (Annotated)splash10-00b9-9000000000-7adf49075b14108e9349View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 25V, Positive (Annotated)splash10-0006-9000000000-576cac3520e069cb3320View in MoNA
LC-MS/MSLC-MS/MS Spectrum - Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9000000000-589800c2ea24ecb7aebfView in MoNA
1D NMR1H NMR SpectrumNot Available
Biological Properties
Cellular Locations
  • Cytoplasm (predicted from logP)
Biofluid Locations
  • Blood
  • Urine
Tissue LocationNot Available
PathwaysNot Available
Normal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
BloodExpected but not QuantifiedNot ApplicableNot AvailableNot Available
Normal
  • Not Applicable
details
UrineDetected and Quantified0.24 +/- 0.36 umol/mmol creatinineInfant (0-1 year old)BothNormal details
Abnormal Concentrations
BiofluidStatusValueAgeSexConditionReferenceDetails
UrineDetected and Quantified2.0 (0.38-3.8) umol/mmol creatinineChildren (1-13 years old)BothShort chain acyl-CoA Dehydrogenase deficiency (SCAD) details
UrineDetected and Quantified1.0 (0.0-2.0) umol/mmol creatinineAdult (>18 years old)BothEthylmalonic encephalopathy details
UrineDetected and Quantified101.0 (2.0-200.0) umol/mmol creatinineChildren (1-13 years old)BothEthylmalonic encephalopathy details
Associated Disorders and Diseases
Disease References
Short Chain Acyl-Coa Dehydrogenase Deficiency
  1. MetaGene [Link]
Ethylmalonic encephalopathy
  1. MetaGene [Link]
Associated OMIM IDsNone
DrugBank IDNot Available
DrugBank Metabolite IDNot Available
Phenol Explorer Compound IDNot Available
Phenol Explorer Metabolite IDNot Available
FoodDB IDFDB022256
KNApSAcK IDNot Available
Chemspider ID79766
KEGG Compound IDNot Available
BioCyc IDNot Available
BiGG IDNot Available
Wikipedia LinkNot Available
NuGOwiki LinkHMDB00808
Metagene LinkHMDB00808
METLIN ID5772
PubChem Compound88412
PDB IDNot Available
ChEBI IDNot Available
References
Synthesis ReferenceBondi, S.; Eissler, F. Lipoproteins and the Fatty Degeneration of Cells. Biochemische Zeitschrift (1910), 23 499-513.
Material Safety Data Sheet (MSDS)Not Available
General References
  1. Tein I, Haslam RH, Rhead WJ, Bennett MJ, Becker LE, Vockley J: Short-chain acyl-CoA dehydrogenase deficiency: a cause of ophthalmoplegia and multicore myopathy. Neurology. 1999 Jan 15;52(2):366-72. [9932958 ]
  2. Bhala A, Willi SM, Rinaldo P, Bennett MJ, Schmidt-Sommerfeld E, Hale DE: Clinical and biochemical characterization of short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jun;126(6):910-5. [7776094 ]
  3. Ito T, Kidouchi K, Sugiyama N, Kajita M, Chiba T, Niwa T, Wada Y: Liquid chromatographic-atmospheric pressure chemical ionization mass spectrometric analysis of glycine conjugates and urinary isovalerylglycine in isovaleric acidemia. J Chromatogr B Biomed Appl. 1995 Aug 18;670(2):317-22. [8548022 ]
  4. Dawson DB, Waber L, Hale DE, Bennett MJ: Transient organic aciduria and persistent lacticacidemia in a patient with short-chain acyl-coenzyme A dehydrogenase deficiency. J Pediatr. 1995 Jan;126(1):69-71. [7815229 ]
  5. Hagen T, Korson MS, Sakamoto M, Evans JE: A GC/MS/MS screening method for multiple organic acidemias from urine specimens. Clin Chim Acta. 1999 May;283(1-2):77-88. [10404733 ]

Enzymes

General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers an acyl group to the N-terminus of glycine and glutamine, although much less efficiently. Can conjugate numerous substrates to form a variety of N-acylglycines, with a preference for benzoyl-CoA over phenylacetyl-CoA as acyl donors. Thereby detoxify xenobiotics, such as benzoic acid or salicylic acid, and endogenous organic acids, such as isovaleric acid.
Gene Name:
GLYAT
Uniprot ID:
Q6IB77
Molecular weight:
18506.33
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers an acyl group to the N-terminus of glutamine. Can use phenylacetyl-CoA as an acyl donor.
Gene Name:
GLYATL1
Uniprot ID:
Q969I3
Molecular weight:
35100.895
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Mitochondrial acyltransferase which transfers the acyl group to the N-terminus of glycine. Conjugates numerous substrates, such as arachidonoyl-CoA and saturated medium and long-chain acyl-CoAs ranging from chain-length C8:0-CoA to C18:0-CoA, to form a variety of N-acylglycines. Shows a preference for monounsaturated fatty acid oleoyl-CoA (C18:1-CoA) as an acyl donor. Does not exhibit any activity toward C22:6-CoA and chenodeoxycholoyl-CoA, nor toward serine or alanine.
Gene Name:
GLYATL2
Uniprot ID:
Q8WU03
Molecular weight:
34277.055
General function:
Involved in glycine N-acyltransferase activity
Specific function:
Acyltransferase which transfers the acyl group to the N- terminus of glycine
Gene Name:
GLYATL3
Uniprot ID:
Q5SZD4
Molecular weight:
32703.3