THE IDENTIFICATION AND THE EXCRETION PATTERN OF ISOVALERYL GLUCURONIDE IN THE URINE OF PATIENTS WITH ISOVALERIC ACIDEMIA

被引：9

作者：

HINE, DG ^{[1
]}

TANAKA, K ^{[1
]}

机构：

[1] YALE UNIV,SCH MED,DEPT HUMAN GENET,NEW HAVEN,CT 06510

来源：

PEDIATRIC RESEARCH | 1984年 / 18卷 / 06期

关键词：

D O I：

10.1203/00006450-198406000-00004

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

引用

页码：508 / 512

页数：5

共 13 条

[1]

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[2]

Dutton G. J., 1966, GLUCURONIC ACID FREE

[3]

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[4] 3-HYDROXYISOHEPTANOIC ACID - A NEW METABOLITE IN ISOVALERIC ACIDEMIA [J].

LEHNERT, W .

CLINICA CHIMICA ACTA, 1981, 113 (01) :101-103

[5] EXCRETION OF N-ISOVALERYLGLUTAMIC ACID IN ISOVALERIC ACIDEMIA [J].

LEHNERT, W .

CLINICA CHIMICA ACTA, 1981, 116 (02) :249-252

[6] GAS-CHROMATOGRAPHY AND MASS-SPECTROMETRY OF SOME TRIMETHYLSILYL DERIVATIVES OF URINARY GLUCURONIDES [J].

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RAINEY, WT .

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[7] DEMONSTRATION OF A SPECIFIC MITOCHONDRIAL ISOVALERYL-COA DEHYDROGENASE-DEFICIENCY IN FIBROBLASTS FROM PATIENTS WITH ISOVALERIC ACIDEMIA [J].

RHEAD, WJ ;

TANAKA, K .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA-BIOLOGICAL SCIENCES, 1980, 77 (01) :580-583

[8] IDENTIFICATION OF BETA-HYDROXYISOVALERIC ACID IN URINE OF A PATIENT WITH ISOVALERIC ACIDEMIA [J].

TANAKA, K ;

ORR, JC ;

ISSELBACHER, KJ .

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[9]

TANAKA K, 1980, CLIN CHEM, V26, P1847

[10] ISOVALERIC ACIDEMIA - A NEW GENETIC DEFECT OF LEUCINE METABOLISM [J].

TANAKA, K ;

BUDD, MA ;

EFRON, ML ;

ISSELBACHER, KJ .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 1966, 56 (01) :236-+

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