Tiglylglycine 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. Tiglylglycine is an intermediate product of the catabolism of isoleucine. An elevated level of tiglylglycine is identified in urine of patients with beta-ketothiolase deficiency or with disorders of propionate metabolism. Tiglyglycine is a biomarker for the consumption of cheese. Tiglyglycine (TG), an intermediate product of the catabolism of isoleucine, is increased in the urine of patients with beta-ketothiolase deficiency or with disorders of propionate metabolism. It is also implicated as a useful diagnostic marker in disorders of the respiratory chain. In the mitochondria, isoleucine is converted to 2-oxo-3-methylvaleric acid vis a transaminase. This is then converted into 2-methylbutyryl-CoA by the BCKADH complex. This is then converted to tiglyl-CoA by SBCAD which can then be made into tiglylglycine via glycine N-acyltransferase.

Metabolism and Physiological Effects of Tiglylglycine References