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二氢硫辛酰胺脱氢酶
命名
系统命名
缩写
识别码
EC編號	1.8.1.4
CAS号	9001-18-7
数据库
IntEnz	IntEnz浏览
BRENDA（英语：BRENDA）	BRENDA入口
ExPASy（英语：ExPASy）	NiceZyme浏览
KEGG	KEGG入口
MetaCyc（英语：MetaCyc）	代谢路径
PRIAM（英语：PRIAM_enzyme-specific_profiles）	概述
PDB	RCSB PDB PDBj PDBe PDBsum
基因本体	AmiGO / EGO
搜索
PMC	相关文献
PubMed	相关文献

二氢硫辛酰胺脱氢酶
二氢硫辛酰胺脱氢酶
	PDB渲染的1zy8
有效结构
PDB	直系同源检索：PDBe, RCSB
PDB查询代码列表
	1ZMC, 1ZMD, 1ZY8, 2F5Z
标识
代号	DLD; DLDH; E3; GCSL; LAD; PHE3
扩展标识	遗传学：238331 鼠基因：107450 同源基因：84 GeneCards: DLD Gene
EC編號	1.8.1.4
基因本体论描述
分子功能	· dihydrolipoyl dehydrogenase activity; · electron carrier activity; · oxidoreductase activity; · lipoamide binding; · flavin adenine dinucleotide binding; · NAD binding;
细胞成分	· cytoplasm; · mitochondrion; · mitochondrial matrix; · flagellum; · acrosomal matrix; · oxoglutarate dehydrogenase complex; · pyruvate dehydrogenase complex;
生物过程	· pyruvate metabolic process; · tricarboxylic acid cycle; · 2-oxoglutarate metabolic process; · mitochondrial electron transport, NADH to ubiquinone; · proteolysis; · lysine catabolic process; · gastrulation; · aging; · branched chain family amino acid catabolic process; · lipoate metabolic process; · regulation of acetyl-CoA biosynthetic process from pyruvate; · cellular nitrogen compound metabolic process; · regulation of membrane potential; · cell redox homeostasis; · sperm capacitation; · dihydrolipoamide metabolic process;
	Sources: Amigo / QuickGO
直系同源体
物种	人类
Entrez	1738
Ensembl	ENSG00000091140
UniProt	P09622
mRNA序列	NM_000108
蛋白序列	NP_000099
基因位置	Chr 7:; 107.53 – 107.57 Mb
PubMed查询	[1]
	查; 论; 编;

二氢硫辛酰胺脱氢酶（EC 1.8.1.4，英語：Dihydrolipoamide dehydrogenase，缩写DLD，又称为线粒体二氢硫辛酸脱氢酶，dihydrolipoyl dehydrogenase, mitochondrial）是一种由人类基因DLD^[1]^[2]^[3]^[4]所编码的黄素蛋白，其作用是将二氢硫辛酰胺脱氢从而转化为氧化型硫辛酰胺。

DLD作为一种线粒体蛋白质，在真核生物的能量代谢中起到重要作用，它至少参与了五种多酶复合体，且为复合体完成反应所必需的组份^[5]。另外，DLD作为一种黄素蛋白氧化還原酶，以FAD为辅基接受质子与电子催化二硫键的形成。

DLD是大小为51千道尔顿亚基的同二聚体，其中每个亚基都与一分子的FAD以共价键的形式相连^[6]。

功能

在哺乳动物的线粒体三羧酸循环中，该酶与丙酮酸脱氢酶和二氢硫辛酰基乙酰基转基酶一起组成中丙酮酸脱氢酶复合体，同时该酶亦是α-酮戊二酸脱氢酶复合体中的一个组分。二氢硫辛酰胺脱氢酶的主要任务是负责将复合物中另一组分硫辛酰胺转乙酰基酶（或二氢硫辛酰琥珀酰转移酶）的辅基二氢硫辛酰胺脱氢从而转化为氧化型硫辛酰胺，该酶也是线粒体甘氨酸剪切体系的组分。

参考文献

^ Entrez Gene: dihydrolipoamide dehydrogenase. （原始内容存档于2010-03-06）.
^ Otulakowski G, Robinson BH. Isolation and sequence determination of cDNA clones for porcine and human lipoamide dehydrogenase. Homology to other disulfide oxidoreductases. J. Biol. Chem. December 1987, 262 (36): 17313–8. PMID 3693355.
^ Pons G, Raefsky-Estrin C, Carothers DJ, Pepin RA, Javed AA, Jesse BW, Ganapathi MK, Samols D, Patel MS. Cloning and cDNA sequence of the dihydrolipoamide dehydrogenase component human alpha-ketoacid dehydrogenase complexes. Proc. Natl. Acad. Sci. U.S.A. March 1988, 85 (5): 1422–6. PMC 279783 . PMID 3278312. doi:10.1073/pnas.85.5.1422.
^ Scherer SW, Otulakowski G, Robinson BH, Tsui LC. Localization of the human dihydrolipoamide dehydrogenase gene (DLD) to 7q31----q32. Cytogenet. Cell Genet. 1991, 56 (3-4): 176–7. PMID 2055113. doi:10.1159/000133081.
^ Babady NE, Pang YP, Elpeleg O, Isaya G. Cryptic proteolytic activity of dihydrolipoamide dehydrogenase. Proceedings of the National Academy of Sciences of the United States of America. 2007, 104 (15): 6158–63. PMC 1851069 . PMID 17404228. doi:10.1073/pnas.0610618104.
^ Ciszak EM, Makal A, Hong YS, Vettaikkorumakankauv AK, Korotchkina LG, Patel MS. How dihydrolipoamide dehydrogenase-binding protein binds dihydrolipoamide dehydrogenase in the human pyruvate dehydrogenase complex. The Journal of Biological Chemistry. 2006, 281 (1): 648–55. PMID 16263718. doi:10.1074/jbc.M507850200.

深入阅读

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