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Identification ofN-acetyl-S-(3-oxo-3-carboxy-n-propyl)cysteine in the urine of a patient with cystathioninuria using LC/APCI-MS

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Journal of Inherited Metabolic Disease

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 syntheticN-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 andR 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 asN-acetyl-S-(3-oxo-3-carboxy-n-propyl)cysteine.

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Authors and Affiliations

  1. Department of Chemistry, Kochi Medical School, Kohasu, Oko-cho, 783, Nankoku-shi, Kochi, Japan

    J. Zhang, K. Sugahara & H. Kodama

  2. Department of Biochemistry, Okayama University, Medical School, Okayama, Japan

    N. Masuoka & T. Ubuka

Authors
  1. J. Zhang
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  2. N. Masuoka
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  3. T. Ubuka
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  4. K. Sugahara
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  5. H. Kodama
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Zhang, J., Masuoka, N., Ubuka, T. et al. Identification ofN-acetyl-S-(3-oxo-3-carboxy-n-propyl)cysteine in the urine of a patient with cystathioninuria using LC/APCI-MS. J Inherit Metab Dis 18, 675–681 (1995). https://doi.org/10.1007/BF02436756

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  • DOI: https://doi.org/10.1007/BF02436756

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