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.
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.
Department of Pediatrics, Kochi Medical School, Japan.
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T
Okada
T
T
Takechi
H
H
Wakiguchi
T
T
Kurashige
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Sugahara
H
H
Kodama
D000592:Amino Acid Metabolism, Inborn Errors / Q000652:urine*; D002851:Chromatography, High Pressure Liquid; D003540:Cystathionine / Q000031:analogs & derivatives* / Q000652:urine; D003545:Cysteine / Q000031:analogs & derivatives; D008401:Gas Chromatography-Mass Spectrometry; D006710:Homocysteine / Q000031:analogs & derivatives; D006801:Humans; D007651:Keto Acids / Q000652:urine*
D007651:Keto Acids; D006710:Homocysteine; D003540:Cystathionine; D003545:Cysteine