Pyroglutamic acid is 5-oxyproline. It is formed by the dehydration between the α-NH2 group and the γ-hydroxyl group of glutamic acid to form an intramolecular amide bond; it can also be formed by the loss of the amido group in the glutamine molecule.
If glutathione synthase deficiency, can cause pyroglutamate, a series of clinical symptoms. Pyroglutamate is a disorder of organic acid metabolism caused by deficiency of glutathione synthase.
Clinical manifestations: Onset 12 to 24 hours after birth, progressive hemolysis, jaundice, chronic metabolic acidosis, mental retardation, etc.; urine contains pyroglutamic acid, lactic acid, α-deoxy-4-glycolacetate lactone. Treatment, symptomatic, pay attention to adjusting diet after one year old.
Complaint
