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Identification of NAc‐HCPC and NAc‐β‐CEC, and qualitative analyses of sulphur amino acids in the urine of a patient with cystathioninuria using liquid chromatography/atmospheric pressure ionization mass spectrometry
Author(s) -
Watanabe Hironobu,
Fujita Yukitoshi,
Sugahara Kazunori,
Kodama Hiroyuki,
Ohmori Shinji
Publication year - 1991
Publication title -
biological mass spectrometry
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.475
H-Index - 121
eISSN - 1096-9888
pISSN - 1052-9306
DOI - 10.1002/bms.1200201005
Subject(s) - chemistry , mass spectrometry , chromatography , cysteine , urine , amino acid , homocysteine , high performance liquid chromatography , sulfur , cystathionine beta synthase , methionine , organic chemistry , biochemistry , enzyme
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‐β‐CEC as N ‐substituted sulphur amino acids were also identified in the urine of the same patient.