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丙酮酸脱氢酶复合体的研究进展及应用
引用本文:崔玉娟,刘晓晴.丙酮酸脱氢酶复合体的研究进展及应用[J].西北师范大学学报,2007,43(6):79-83.
作者姓名:崔玉娟  刘晓晴
作者单位:首都师范大学生命科学学院,北京100037
摘    要:综述了丙酮酸脱氢酶复合体(PDHc)的组成、结构功能、调控机制、缺陷疾病及在医学和农业中的应用.丙酸酸脱氢酶系是定位在线粒体中的多酶复合体,它是由丙酮酸脱氢酶(E1)、二氢硫辛酸乙酰转移酶(E2)、二氢硫辛酸脱氢酶(E3)和一些辅助因子组成.高等生物中PDHc活性的调节主要是通过对其E1的磷酸化和去磷酸化来实现的,而细菌的PDHc的活性主要是通过别构效应来调节,PDHc缺陷导致代谢障碍,组织受损.

关 键 词:丙酮酸脱氢酶复合体(PDHc)  调控机制  缺陷疾病
文章编号:1001-988X(2007)06-0079-05
修稿时间:2007年9月14日

Development and application of pyrurate dehydrogenase complex
CUI Yu-juan,LIU Xiao-qing.Development and application of pyrurate dehydrogenase complex[J].Journal of Northwest Normal University Natural Science (Bimonthly),2007,43(6):79-83.
Authors:CUI Yu-juan  LIU Xiao-qing
Abstract:The article reviewes the PDHc briefly in structure,functional,activity regulation,deficiency,application in medicine and agriculture.PDHc is a mitochondrial multienzyme complex including pyruvate dehydrogenase(E1),dihydrolipoamide acetyltransferase(E2),dihydrolipoamide dehydrogenase(E3),and some cofactor and protein X.Through the phosphorylation and dephosphorylation of the E1(pyruvate dehydrogenase,PDH)of the PDHc,the activity of mammalian pyruvate dehydrogenase complex has been regulated.While the allosteric control is the main regulatory mechanism in the bacterial.If the PDHc is deficient,it may induce holdback of metabolism and the organism may be suffered.
Keywords:pyruvate dehydrogenase complex(PDHc)  regulation mechanism  deficiency
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