Acylglycines result from the transesterification of acyl-CoA esters with glycine through the action of the enzyme glycine-N-acylase. In defects of the mitochondrial amino acid and fatty acid metabolism where accumulation of acyl-CoA esters occurs, increased urinary acylglycine excretion is directly related to the intramitochondrial accumulation of the corresponding acyl-CoA esters.
- acetylglycine - [13C2]acetylglycine
- propionylglycine - [3,3,3-2H3]propionylglycine
- butyrylglycine - [4,4,4-2H3]butyrylglycine
- isobutyrylglycine - [2H7]isobutyrylglycine
- valerylglycine - [5,5,5-2H3]valerylglycine
- isovalerylglycine - [2H9]isovalerylglycine
- 2-methylbutyrylglycine - [2H9]2-methylbutyrylglycine
- hexanoylglycine - [6,6,6-2H3]hexanoylglycine
- phenylpropionylglycine - [2H5]phenylpropionylglycine
Synthesis and application
CG Costa, WS Guérand, EA Struys, U Holwerda, HJ ten Brink, I Tavares de Almeida, M Duran, C Jakobs. Quantitative analysis of urinary acylglycines for the diagnosis of ß-oxidation defects using GC-NCI-MS. J Pharm Biomed Anal 21 (2000) 1215-1224.