Excretion of alpha-keto-delta-guanidinovaleric acid and its cyclic form in patients with hyperargininemia.
alpha-Keto-delta-guanidinovaleric acid, in equilibrium with its cyclic form, has been identified in the urines of three sisters affected with hyperargininemia. Identification was achieved on the basis of its similarity with the enzymatically synthesized compounds.
D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D001120:Arginine / Q000097:blood*; D005260:Female; D006146:Guanidines / Q000652:urine*; D006801:Humans; D007651:Keto Acids / Q000652:urine*; D014631:Valerates / Q000652:urine
D006146:Guanidines; D007651:Keto Acids; D014631:Valerates; C022249:alpha-keto-delta-guanidinovaleric acid; D001120:Arginine