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

被引：19

作者：

OKADA, T

TAKECHI, T

WAKIGUCHI, H

KURASHIGE, T

SUGAHARA, K

KODAMA, H

机构：

[1] KOCHI MED SCH,DEPT CHEM,NANKOKU,KOCHI 783,JAPAN

[2] KOCHI MED SCH,DEPT PEDIAT,NANKOKU,KOCHI 783,JAPAN

来源：

ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS | 1993年 / 305卷 / 02期

关键词：

D O I：

10.1006/abbi.1993.1436

中图分类号：

Q5 [生物化学]; Q7 [分子生物学];

学科分类号：

071010 ; 081704 ;

摘要：

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 μg ± 35.9/g of creatinine. The cystathioninuric patient excreted 20 times more cystathionine mono-oxo acids in the urine than healthy subjects. © 1993 Academic Press, Inc.

引用

页码：385 / 391

页数：7

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