Trans-Cinnamoylglycine is one component of the Acylglycines panel.
Acylglycines are an important class of metabolites that are used in the diagnosis of several organic acidurias and mitochondrial fatty acid oxidation disorders.
Acylglycines are glycine conjugates of acyl-CoA species, which occur as normal intermediates of amino acid and fatty acid metabolism. In abnormal concentrations, acylglycines are biochemical markers of selected inborn errors of metabolism (IEM). Analysis of acylglycines is useful for the diagnosis and monitoring for specific fatty acid oxidation disorders and organic acidurias, although is recommended to use this testing in conjunction with urine organic acids and plasma acylcarnitines testing in order to establish a diagnosis.
In particular, acylglycine analysis is more sensitive and specific for the identification of asymptomatic patients and those who may experience mild or intermittent biochemical phenotypes that could be missed by organic acid analysis alone.
Diagnosis and monitoring for patients affected with 1 of the following inborn errors of metabolism:
Fatty Acid Oxidation Disorders
-Glutaric acidemia type II
-Medium-chain 3-ketoacyl-CoA thiolase (MCKAT) deficiency
-Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency
-Short chain acyl-CoA dehydrogenase (SCAD) deficiency
-2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (2M3HBD) deficiency
-2-Methylbutyryl-CoA dehydrogenase deficiency
-3-Methylcrotonyl-CoA carboxylase deficiency
-Aminoacylase 1 deficiency
-Glutaryl-CoA dehydrogenase deficiency
-Isobutyryl-CoA dehydrogenase (IBD) deficiency
-Isovaleryl-CoA dehydrogenase deficiency
-Multiple carboxylase deficiency
Costa CG, Guerand WS, Struys EA, Holwerda U, ten Brink HJ, Tavares de Almeida I, Duran M, Jakobs C. Quantitative analysis of urinary acylglycines for the diagnosis of beta-oxidation defects using GC-NCI-MS. J Pharm Biomed Anal. 2000 Jan;21(6):1215-24. doi: 10.1016/s0731-7085(99)00235-6. PMID: 10708405.
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