THIAMINE RESPONSE IN MAPLE SYRUP URINE DISEASE

被引：20

作者：

FERNHOFF, PM ^{[1
]}

LUBITZ, D ^{[1
]}

DANNER, DJ ^{[1
]}

DEMBURE, PP ^{[1
]}

SCHWARTZ, HP ^{[1
]}

HILLMAN, R ^{[1
]}

BIER, DM ^{[1
]}

ELSAS, LJ ^{[1
]}

机构：

[1] WASHINGTON UNIV,SCH MED,DEPT PEDIAT,DIV METAB,ST LOUIS,MO 63110

来源：

PEDIATRIC RESEARCH | 1985年 / 19卷 / 10期

关键词：

D O I：

10.1203/00006450-198510000-00012

中图分类号：

R72 [儿科学];

学科分类号：

100202 ;

摘要：

引用

页码：1011 / 1016

页数：6

共 22 条

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[3]

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[6] THIAMINE INCREASES SPECIFIC ACTIVITY OF HUMAN LIVER BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE [J].

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[7] INVIVO AND INVITRO RESPONSE OF HUMAN BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE TO THIAMINE AND THIAMINE PYROPHOSPHATE [J].

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[8] EFFECTS OF THIAMINE IN A PATIENT WITH A VARIANT FORM OF BRANCHED-CHAIN KETOACIDURIA [J].

DURAN, M ;

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WADMAN, SK ;

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KLEIJER, WJ .

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[9] MAPLE SYRUP URINE DISEASE - COENZYME FUNCTION AND PRENATAL MONITORING [J].

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[10] THE ROLE OF THIAMIN IN MAPLE SYRUP URINE DISEASE [J].

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ANNALS OF THE NEW YORK ACADEMY OF SCIENCES, 1982, 378 (MAR) :404-421

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