Identification of new cystathionine mono-oxo acids, S-(3-oxo-3-carboxy-n-propyl) cysteine and S-(2-oxo-2-carboxyethyl) homocysteine, in the urine of a patient with cystathioninuria.
Identification of new cystathionine mono-oxo acids, S-(3-oxo-3-carboxy-n-propyl) cysteine and S-(2-oxo-2-carboxyethyl) homocysteine, in the urine of a patient with cystathioninuria.
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胱硫醚尿症患者尿液中新的胱硫醚单含氧酸、S-(3-氧代-3-羧基-正丙基)半胱氨酸和 S-(2-氧代-2-羧乙基)高半胱氨酸的鉴定。
DOI：
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发表时间：
1993
影响因子：
3.9
通讯作者：
H. Kodama
中科院分区：
文献类型：
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作者：
T. Okada;T. Takechi;H. Wakiguchi;T. Kurashige;K. Sugahara;H. Kodama
新的胱硫醚单含氧酸，S-（3-氧代-3-羧基-正丙基）半胱氨酸和S-（2-氧代-2-羧乙基）高半胱氨酸，和环状氨基酸，胱硫醚酮亚胺，已被发现在尿中的胱硫醚尿症患者使用液相色谱-质谱与大气压电离接口系统和氨基酸分析仪。为了确定这些胱硫醚单含氧酸和胱硫醚酮亚胺，我们利用异硫氰酸苯酯-酮亚胺相互作用产物在380 nm处的选择性吸光度。在胱硫醚尿患者和6名健康受试者的尿样中发现的这些化合物的总浓度分别为3611.3和148.4微克+/- 35.9/g肌酐。胱硫醚尿患者尿液中排出的胱硫醚单含氧酸是健康受试者的20倍。
Novel cystathionine mono-oxo acids, S-(3-oxo-3-carboxy-n-propyl) cysteine and S-(2-oxo-2-carboxyethyl) homocysteine, and cyclic amino acid, cystathionine ketimine, have been detected in the urine of a patient with cystathioninuria using liquid chromatography-mass spectrometry with an atmospheric pressure ionization interface system and an amino acid analyzer. To determine these cystathionine mono-oxo acids and cystathionine ketimine we took advantage of the selective absorbance at 380 nm of the phenylisothiocyanate-ketimine interaction product. The total concentrations of these compounds found in the urine samples of a cystathioninuric patient and six healthy subjects were respectively 3611.3 and 148.4 micrograms +/- 35.9/g of creatinine. The cystathioninuric patient excreted 20 times more cystathionine mono-oxo acids in the urine than healthy subjects.