N-Butyrylglycine
N-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, resliting in the loss of hydrogen bonding donors. Polymers of these compounds are called Peptoids.
Structure for HMDB00808 (N-Butyrylglycine)
C6H11NO3
145.1564
145.073893223
2-butanamidoacetic acid
butyrylglycine
20208-73-5
WPSSBBPLVMTKRN-UHFFFAOYSA-N
This compound belongs to the class of chemical entities 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.
Chemical entities
Organic compounds
Organic acids and derivatives
Carboxylic acids and derivatives
N-acyl-alpha amino acids
Aliphatic acyclic compounds
Not Available
Detected and Quantified
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Solid
LC-MS/MS Spectrum – Quattro_QQQ 10V, Positive (Annotated)splash10-00b9-9000000000-7adf49075b14108e9349View in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 25V, Positive (Annotated)splash10-0006-9000000000-576cac3520e069cb3320View in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 40V, Positive (Annotated)splash10-0006-9000000000-589800c2ea24ecb7aebfView in MoNA
1H NMR SpectrumNot Available
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Not Available
Normal
- MetaGene [Link]
- MetaGene [Link]
None
Not Available
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FDB022256
Not Available
79766
Not Available
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HMDB00808
HMDB00808
5772
88412
Not Available
Not Available
- 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. [PubMed:9932958 ]
- 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. [PubMed:7776094 ]
- 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. [PubMed:8548022 ]
- 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. [PubMed:7815229 ]
- 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. [PubMed: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