Glycine N-acyltransferase (GLYAT)

Glycine N-acyltransferase (GLYAT) is a mitochondrial enzyme that catalyzes the conjugation of glycine with acyl-CoA thioesters, predominantly benzoyl-CoA, resulting in the formation of N-acylglycines such as hippuric acid. This reaction is a key step in the phase II detoxification pathway for both endogenous metabolites and xenobiotics, enabling their excretion. GLYAT displays substrate preference for benzoyl-CoA but can conjugate a broad spectrum of acyl-CoAs. Deficiency or genetic variation in GLYAT can impact the metabolism of compounds like benzoic acid and is relevant to disorders such as isovaleric acidemia and risk of toxicity from food preservatives or salicylate drugs.

Other names

Acyl-CoA:glycine N-acyltransferaseGlycine acyltransferaseGlycine-N-acylaseACGNATEC 2.3.1.13

Detoxification through formation of N-acylglycines, especially conjugating glycine to acyl-CoA thioesters (most notably benzoyl-CoA), facilitating urinary excretion