ALTERED KINETIC-PROPERTIES OF THE BRANCHED-CHAIN ALPHA-KETO ACID DEHYDROGENASE COMPLEX DUE TO MUTATION OF THE BETA-SUBUNIT OF THE BRANCHED-CHAIN ALPHA-KETO ACID DECARBOXYLASE (E1) COMPONENT IN LYMPHOBLASTOID-CELLS DERIVED FROM PATIENTS WITH MAPLE SYRUP URINE DISEASE

被引：52

作者：

INDO, Y ^{[1
]}

KITANO, A ^{[1
]}

ENDO, F ^{[1
]}

AKABOSHI, I ^{[1
]}

MATSUDA, I ^{[1
]}

机构：

[1] KUMAMOTO UNIV,SCH MED,DEPT PEDIAT,HONJO 1-1-1,KUMAMOTO 860,JAPAN

来源：

JOURNAL OF CLINICAL INVESTIGATION | 1987年 / 80卷 / 01期

关键词：

D O I：

10.1172/JCI113064

中图分类号：

R-3 [医学研究方法]; R3 [基础医学];

学科分类号：

1001 ;

摘要：

引用

页码：63 / 70

页数：8

共 46 条

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