Activity of the glycine cleavage system in hyperammonemia treated with benzoate.

We investigated levels of the glycine cleavage system in livers of spf-fur mutant mice with ornithine transcarbamylase (OTC) deficiency treated with sodium benzoate. The activities of the glycine cleavage system in benzoate-treated spf/Y males and the control mice livers are not significantly different. We examined plasma folate level and Vit. B6 status in a patient with OTC deficiency during the therapy with benzoate. Plasma folate level and vitamin B6 status during the therapy period and during control period are not different and these data were within normal ranges. The observation suggests that the glycine cleavage system is not the candidate for the increase of the de novo synthesis of glycine in hyperammonemic patients treated with benzoate.