SUBERYLGLYCINE EXCRETION IN URINE FROM A PATIENT WITH DICARBOXYLIC ACIDURIA

被引：120

作者：

GREGERSEN, N

LAURITZEN, R

RASMUSSEN, K

机构：

[1] UNIV AARHUS, AARHUS KOMMUNE HOSP, DEPT CLIN CHEM, BIOCHEM RES UNIT, DK-8000 AARHUS, DENMARK

[2] AALBORG HOSP N, DEPT PEDIAT, DK-9000 AALBORG, DENMARK

来源：

CLINICA CHIMICA ACTA | 1976年 / 70卷 / 03期

关键词：

D O I：

10.1016/0009-8981(76)90355-7

中图分类号：

R446 [实验室诊断]; R-33 [实验医学、医学实验];

学科分类号：

1001 ;

摘要：

Suberylglycine (HOOC(CH2)6CONHCH2COOH) was found in the urine from a patient with C6-C10-.omega.-dicarboxylic aciduria and unexplained episodes of lethargy and unconsciousness. The total excretion of adipic, suberic and sebacic acid ranged from 0.77-1.3 mg/mg creatinine after episodes of acute attack of the disease. Suberylglycine, identified by gas chromatography/mass spectrometry, was repeatedly found in the urine samples. The amount of this conjugate ranged from 0.2-0.5 mg/mg creatinine. The precursors of the dicarboxylic acids may be long chain monocarboxylic acids, oxidized through .omega.- and .beta.-oxidation to adipic, suberic and sebacic acid. Suberylglycine is subsequently formed by glycine-N-acylase-catalyzed conjugation.

引用

页码：417 / 425

页数：9

共 26 条

[1] ISOVALERIC-ACIDEMIA - IDENTIFICATION OF ISOVALERATE, ISOVALERYLGLYCINE, AND 3-HYDROXYISOVALERATE IN URINE OF A PATIENT PREVIOUSLY REPORTED AS HAVING BUTYRIC AND HEXANOIC ACIDEMIA [J].