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Identification of N ‐acetyl‐ S ‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine in the urine of a patient with cystathioninuria using LC/APCI‐MS
Author(s) -
Zhang J.,
Masuoka N.,
Ubuka T.,
Sugahara K.,
Kodama H.
Publication year - 1995
Publication title -
journal of inherited metabolic disease
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.462
H-Index - 102
eISSN - 1573-2665
pISSN - 0141-8955
DOI - 10.1007/bf02436756
Subject(s) - atmospheric pressure chemical ionization , chemistry , urine , metabolite , mass spectrometry , chromatography , cysteine , liquid chromatography–mass spectrometry , cystathionine beta synthase , high performance liquid chromatography , chemical ionization , ion , ionization , biochemistry , organic chemistry , enzyme
Summary A new cystathionine metabolite has been identified in the urine of a patient with cystathioninuria using liquid chromatography‐mass spectrometry with an atmospheric pressure chemical ionization interface system (LC/APCI‐MS). By this method a very intense quasi‐molecular ion was observed as a base peak of synthetic N ‐acetyl‐ S ‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine (NAc‐OCPC). The quasimolecular ion [M+H] + of NAc‐OCPC observed in the urine of a patient with cystathioninuria was the same as that of the authentic compound ( m/z 264). The retention time and R f value on paper chromatography of the synthetic compound were the same as those of the urinary compound from the patient with cystathioninuria. From these results, this new cystathionine metabolite was identified as N ‐acetyl‐ S ‐(3‐oxo‐3‐carboxy‐n‐propyl)cysteine.