Glutarylglycine
Glutarylglycine 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. Glutarylglycine is involved in lysine metabolism. An elevated level of glutarylglycine occurs in patients with glutaric acidemia type II, which is an autosomal recessive inborn error of metabolism due to a mitochondrial respiratory electron chain transport defect. (http://www.pediatricneuro.com/alfonso/pg75.htm).
Structure for HMDB00590 (Glutarylglycine)
C7H11NO5
189.1659
189.063722467
4-[(carboxymethyl)carbamoyl]butanoic acid
4-(carboxymethylcarbamoyl)butanoic acid
17686-38-3
NBLKTRLPZFCMJG-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
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Expected but not Quantified
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Solid
LC-MS/MS Spectrum – Quattro_QQQ 10V, Positive (Annotated)splash10-004i-4900000000-5203bd37b71d2695326aView in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 25V, Positive (Annotated)splash10-0092-9200000000-aff109666c05e024b5a8View in MoNA
LC-MS/MS Spectrum – Quattro_QQQ 40V, Positive (Annotated)splash10-004i-9300000000-b1ef334d6ee7b6566ef3View in MoNA
1H NMR SpectrumNot Available
[1H,13C] 2D NMR SpectrumNot Available
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None
None
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FDB022131
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19712034
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HMDB00590
HMDB00590
5569
23592950
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Product: Pranlukast (hemihydrate)
- Ohie T, Fu X, Iga M, Kimura M, Yamaguchi S: Gas chromatography-mass spectrometry with tert.-butyldimethylsilyl derivation: use of the simplified sample preparations and the automated data system to screen for organic acidemias. J Chromatogr B Biomed Sci Appl. 2000 Sep 1;746(1):63-73. [PubMed:11048741 ]
- Gron I, Gregersen N, Kolvraa S, Rasmussen K: Formation of N-dicarboxyl-mono-glycines catalyzed by glycine-N-acylase. Biochem Med. 1978 Feb;19(1):133-48. [PubMed:623642 ]
- Gregersen N, Kolvraa S, Rasmussen K, Gron I: Demonstration of N-dicarboxyl-mono-glycines in dicarboxylic acidurias by mass fragmentography. Clin Chim Acta. 1977 Jul 15;78(2):173-82. [PubMed:884855 ]
- Bonafe L, Troxler H, Kuster T, Heizmann CW, Chamoles NA, Burlina AB, Blau N: Evaluation of urinary acylglycines by electrospray tandem mass spectrometry in mitochondrial energy metabolism defects and organic acidurias. Mol Genet Metab. 2000 Apr;69(4):302-11. [PubMed:10870848 ]
- Gregersen N, Gron I, Rasmussen K, Kolvraa S: Gas chromatographic mass spectrometric identification of N-dicarboxylmonoglycines. Biomed Mass Spectrom. 1978 Jan;5(1):80-3. [PubMed:623898 ]
- (). Asai, Kiyofumi. Urinary glutarylglycine in a patient with glutaric aciduria type I. Quantification of urinary glutarylglycine by a carboxylic acid analyzer and its clinical application. Nagoya-shiritsu Daigaku Igakkai Zasshi (1989), 40(4), 743-58. .
- (). Curtius, T., Hechtenberg, Wihelm. Synthesis of -aminobutyric acid from glutarylglycine ester. Journal fuer Praktische Chemie (Leipzig) (1923), 105 319-26. .
- (). Fujita, A. Synthesis of anemoninic acid. Yakugaku Zasshi (1923), No. 492 67-75. .
- (). Paul, L., Dittmar, A., Rusch, Christel. Synthesis of maleoyl- and glutarylglycyl chlorides. Chemische Berichte (1967), 100(8), 2757-60. .
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