3-Aminopiperid-2-one, an unusual metabolite in the urine of a patient with hyperammonaemia, hyperornithinaemia and homocitrullinuria.
The urine of a patient with a disorder characterized by hyperammonaemia, hyperornithinaemia and homocitrullinuria contains abnormal amounts of 3-aminopiperid-2-one. The properties of this compound closely resemble those ascribed previously (Gordon, B.A., Gatfield, P.D. and Taller, E. (1977) Clin. Biochem. 10, 78-82) to ornithine methyl ester. The excretion of the aminopiperidone ranged from 130-1050 micronmol/g creatinine.
D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D000641:Ammonia / Q000097:blood*; D002849:Chromatography, Gas; D002852:Chromatography, Ion Exchange; D002956:Citrulline / Q000031:analogs & derivatives* / Q000652:urine; D004586:Electrophoresis; D006801:Humans; D013058:Mass Spectrometry; D009952:Ornithine / Q000097:blood*; D010880:Piperidines / Q000652:urine*; D010881:Piperidones / Q000652:urine*
D010880:Piperidines; D010881:Piperidones; D002956:Citrulline; D000641:Ammonia; D009952:Ornithine