描述
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) [HMDB]
InChI标识符
InChI=1S/C7H11NO5/c9-5(8-4-7(12)13)2-1-3-6(10)11/h1-4H2,(H,8,9)(H,10,11)(H,12,13)