二氫硫辛酰胺脫氫酶

二氫硫辛酰胺脫氫酶
命名
系統(tǒng)命名
縮寫
識別碼
EC編號	1.8.1.4
CAS號	9001-18-7
數(shù)據(jù)庫
IntEnz	IntEnz瀏覽
BRENDA	BRENDA入口
ExPASy	NiceZyme瀏覽
KEGG	KEGG入口
MetaCyc	代謝路徑
PRIAM	概述
PDB	RCSB PDB PDBe PDBsum
基因本體	AmiGO / EGO
搜索
PMC	相關(guān)文獻
PubMed	相關(guān)文獻

二氫硫辛酰胺脫氫酶
	; PDB渲染的1zy8
有效結(jié)構(gòu)
PDB	直系同源檢索：PDBe, RCSB
PDB查詢代碼列表
	1ZMC, 1ZMD, 1ZY8, 2F5Z
標(biāo)識
代號	DLD; DLDH; E3; GCSL; LAD; PHE3
擴展標(biāo)識	遺傳學(xué)：238331 鼠基因：107450 同源基因：84 GeneCards: DLD Gene
EC編號	1.8.1.4
基因本體論描述
分子功能	· dihydrolipoyl dehydrogenase activity; · electron carrier activity; · oxidoreductase activity; · lipoamide binding; · flavin adenine dinucleotide binding; · NAD binding;
細胞成分	· cytoplasm; · mitochondrion; · mitochondrial matrix; · flagellum; · acrosomal matrix; · oxoglutarate dehydrogenase complex; · pyruvate dehydrogenase complex;
生物過程	· 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
直系同源體
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A+醫(yī)學(xué)百科 >> 二氫硫辛酰胺脫氫酶

二氫硫辛酰胺脫氫酶（EC 1.8.1.4，英語：Dihydrolipoamide dehydrogenase，縮寫DLD，又稱為線粒體二氫硫辛酸脫氫酶，dihydrolipoyl dehydrogenase, mitochondrial）是一種由人類基因DLD^[1]^[2]^[3]^[4]所編碼的黃素蛋白，其作用是將二氫硫辛酰胺脫氫從而轉(zhuǎn)化為氧化型硫辛酰胺。

DLD作為一種線粒體蛋白質(zhì)，在真核生物的能量代謝中起到重要作用，它至少參與了五種多酶復(fù)合體，且為復(fù)合體完成反應(yīng)所必需的組份^[5]。另外，DLD作為一種黃素蛋白氧化還原酶，以FAD為輔基接受質(zhì)子與電子催化二硫鍵的形成。

DLD是大小為51千道爾頓亞基的同二聚體，其中每個亞基都與一分子的FAD以共價鍵的形式相連^[6]。

硫辛酰胺

二氫硫辛酰胺

功能

在哺乳動物的線粒體三羧酸循環(huán)中，該酶與丙酮酸脫氫酶和二氫硫辛酰基乙酰基轉(zhuǎn)基酶一起組成中丙酮酸脫氫酶復(fù)合體，同時該酶亦是α-酮戊二酸脫氫酶復(fù)合體中的一個組分。二氫硫辛酰胺脫氫酶的主要任務(wù)是負責(zé)將復(fù)合物中另一組分硫辛酰胺轉(zhuǎn)乙酰基酶（或二氫硫辛酰琥珀酰轉(zhuǎn)移酶）的輔基二氫硫辛酰胺脫氫從而轉(zhuǎn)化為氧化型硫辛酰胺，該酶也是線粒體甘氨酸剪切體系的組分。

參考文獻

↑ Entrez Gene: dihydrolipoamide dehydrogenase.
↑ 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. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC279783/ 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. doi:10.1073/pnas.85.5.1422. PMID 3278312. PMC 279783.
↑ 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. doi:10.1159/000133081. PMID 2055113.
↑ Babady NE, Pang YP, Elpeleg O, Isaya G. [http//www.ncbi.nlm.nih.gov/pmc/articles/PMC1851069/ Cryptic proteolytic activity of dihydrolipoamide dehydrogenase]. Proceedings of the National Academy of Sciences of the United States of America. 2007, 104 (15): 6158–63. doi:10.1073/pnas.0610618104. PMID 17404228. PMC 1851069.
↑ 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. doi:10.1074/jbc.M507850200. PMID 16263718.

深入閱讀

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