Identification of NAc-HCPC and NAc-beta-CEC, and qualitative analyses of sulphur amino acids in the urine of a patient with cystathioninuria using liquid chromatography/atmospheric pressure ionization mass spectrometry.
Standard sulphur amino acids and various cystathionine metabolites in the urine of a patient with cystathioninuria were analysed using liquid chromatography/mass spectrometry with an atmospheric pressure ionization interface system. Very intense quasi-molecular ions ([M + H]+) of synthetic cystathionine, N-monoacetylcystathionine, perhydro-1,4-thiazepine-3,5-dicarboxylic acid, S-(3-hydroxy-3-carboxy-n-propyl)cysteine, S-(2-carboxyethyl) cysteine, S-(2-hydroxy-2-carboxyethyl)homocysteine, S-(carboxymethyl)homocysteine, N-acetyl-S-(3-hydroxy-3-carboxy-n-propyl)cysteine and N-acetyl-S-(2-carboxyethyl)cysteine were observed by this method. Quasi-molecular ions ([M + H]+) of these sulphur amino acids were observed in the urine sample of the patient with cystathioninuria, and N-acetyl-HCPC and N-acetyl-beta-CEC as N-substituted sulphur amino acids were also identified in the urine of the same patient.
Watanabe
H
H
Department of Urology, Kochi Medical School, Japan.
Fujita
Y
Y
Sugahara
K
K
Kodama
H
H
Ohmori
S
S
D000111:Acetylcysteine / Q000031:analogs & derivatives* / Q000652:urine; D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D000603:Amino Acids, Sulfur / Q000652:urine*; D002853:Chromatography, Liquid; D003540:Cystathionine / Q000652:urine*; D005260:Female; D006801:Humans; D066298:In Vitro Techniques; D013058:Mass Spectrometry
D000603:Amino Acids, Sulfur; C073257:S-(3-hydroxy-3-carboxy-n-propyl)-N-acetylcysteine; D003540:Cystathionine; C073258:S-(2-carboxyethyl)-N-acetylcysteine; D000111:Acetylcysteine