IDENTIFICATION OF N-ACETYL-ALPHA-AMINOADIPIC ACID IN THE URINE OF A PATIENT WITH ALPHA-AMINOADIPIC AND ALPHA-KETOADIPIC ACIDURIA

被引：11

作者：

TAKECHI, T ^{[1
]}

OKADA, T ^{[1
]}

WAKIGUCHI, H ^{[1
]}

MORITA, H ^{[1
]}

KURASHIGE, T ^{[1
]}

SUGAHARA, K ^{[1
]}

KODAMA, H ^{[1
]}

机构：

[1] KOCHI UNIV,DEPT CHEM,NANKOKU,KOCHI 783,JAPAN

来源：

JOURNAL OF INHERITED METABOLIC DISEASE | 1993年 / 16卷 / 01期

关键词：

D O I：

10.1007/BF00711325

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

A 3.5-year-old Japanese boy with a mild speech disturbance excreted a large amount of alpha-aminoadipic acid into the urine. The amino acid analysis using an amino acid analyser confirmed the presence of alpha-aminoadipic acid in both urine and plasma. We detected alpha-aminoadipic acid in the hydrolysate of the effluent and washwater fraction through cation exchange resin. This suggested the presence of acetylated derivatives and we identified N-acetyl-alpha-aminoadipic acid using liquid chromatography-atmospheric pressure ionization mass spectrometry (LC-API-MS). The concentrations of alpha-aminoadipic acid and N-acetyl-alpha-aminoadipic acid in the urine of a patient with alpha-aminoadipic aciduria were 376.9 nmol/mg creatinine and 18.1 nmol/mg creatinine, respectively. We also detected alpha-ketoadipic acid in the urine of this patient using LC-API-MS.

引用

页码：119 / 126

页数：8

共 15 条

[1]

Casey R E, 1978, J Inherit Metab Dis, V1, P129, DOI 10.1007/BF01805581

[2] A PATIENT WITH ALPHA-KETOADIPIC AND ALPHA-AMINOADIPIC ACIDURIA [J].