Suberylglycine excretion in the urine from a patient with dicarboxylic aciduria.
Suberylglycine (HOOC(CH2)6CONHCH2COOH) was found in the urine from a patient with C6-C10-omega-dicarboxylic aciduria and unexplained episodes of lethargy and unconsciousness. The total excretion of adipic, suberic and sebacic acid ranged from 0.77 to 1.3 mg/mg creatinine after episodes of acute attack of the disease. Suberylglycine, identified by gas chromatography/mass spectrometry, was repeatedly found in the urine samples. The amount of this conjugate ranged from 0.2 to 0.5 mg/mg creatinine. The precursors of the dicarboxylic acids are suggested to be long chain monocarboxylic acids, oxidized through omega- and beta-oxidation to adipic, suberic and sebacic acid. Suberylglycine is subsequently formed by glycine-N-acylase catalyzed conjugation.
D000272:Adipates / Q000652:urine; D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D003404:Creatinine / Q000652:urine; D003998:Dicarboxylic Acids / Q000652:urine*; D005260:Female; D008401:Gas Chromatography-Mass Spectrometry; D005998:Glycine / Q000031:analogs & derivatives* / Q000652:urine; D006801:Humans; D007223:Infant; D008297:Male
D000272:Adipates; D003998:Dicarboxylic Acids; C013264:suberylglycine; D003404:Creatinine; D005998:Glycine