Center for Bioinformatics
Oxidoreductases | Transferases | Hydrolases | Lyases | Isomerases | Ligases

Basic Information

Enzyme Number

1.2.4.2/2.3.1.61/1.8.1.4

Official Name

dihydrolipoyllysine-residue succinyltransferase

Name from literature

oxoglutarate dehydrogenase complex

Pathway from literature

citric acid cycle

Pathway from KEGG

Carbohydrate Metabolism; Pyruvate metabolism; map00620

Carbohydrate Metabolism; Glycolysis / Gluconeogenesis; map00010

Carbohydrate Metabolism; Citrate cycle (TCA cycle); map00020

Amino Acid Metabolism; Tryptophan metabolism; map00380

Amino Acid Metabolism; Glycine, serine and threonine metabolism; map00260

Amino Acid Metabolism; Valine, leucine and isoleucine degradation; map00280

Amino Acid Metabolism; Lysine degradation; map00310

Amino Acid Metabolism; Alanine and aspartate metabolism; map00252

Organisms

Rat (10116)

Genome localization

14q21[360975 ], 6q16[298942 ], 16p16[290566 ],

Comments

A multimer (24-mer) of this enzyme forms the core of the multienzyme complex, and binds tightly both EC 1.2.4.2, oxoglutarate dehydrogenase (succinyl-transferring) and EC 1.8.1.4, dihydrolipoyl dehydrogenase. The lipoyl group of this enzyme is reductively succinylated by EC 1.2.4.2, and the only observed direction catalysed by EC 2.3.1.61 is that where this succinyl group is passed to coenzyme A.

Rate-limiting Description

"Diminished activity of the alpha-ketoglutarate dehydrogenase complex (KGDHC), a key and arguably rate-limiting enzyme of the Krebs cycle, occurs in these disorders and may underlie decreased brain metabolism." (14515360)

"Oxoglutarate dehydrogenase shows the lowest activities along the whole nephron and appears to catalyze the rate-limiting step of the tricarboxylic acid cycle." (7155797)

"NAD+-isocitrate dehydrogenase and oxoglutarate dehydrogenase appeared to be rate-limiting in the oxidation of threo-Ds-isocitrate and oxoglutarate by uncoupled mitochondria from brown adipose tissue of cold-adapted rats." (6778477)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [Q6P6R2 ];
GO:0031966 (C) mitochondrial membrane [Q5XI78 ];
GO:0006096 (P) glycolysis [Q4KLP0, Q5XI78 ];
GO:0005739 (C) mitochondrion [Q4KLP0, Q01205 ];
GO:0045252 (C) oxoglutarate dehydrogenase complex [Q01205 ];
GO:0004148 (F) dihydrolipoyl dehydrogenase activity [Q6P6R2 ];
GO:0031405 (F) lipoic acid binding [Q01205 ];
GO:0006099 (P) tricarboxylic acid cycle [Q01205 ];
GO:0005759 (C) mitochondrial matrix [Q6P6R2, Q5XI78 ];
GO:0009055 (F) electron carrier activity [Q6P6R2 ];
GO:0045454 (P) cell redox homeostasis [Q6P6R2 ];
GO:0004591 (F) oxoglutarate dehydrogenase (succinyl-transf... [Q4KLP0, Q5XI78 ];
GO:0030976 (F) thiamin pyrophosphate binding [Q4KLP0, Q5XI78 ];
GO:0055114 (P) oxidation reduction [Q4KLP0, Q6P6R2, Q5XI78 ];
GO:0004149 (F) dihydrolipoyllysine-residue succinyltransfe... [Q01205 ];

Subcellular localization

Localization

mitochondrion [Q4KLP0, Q6P6R2, Q01205, Q5XI78 ];

Links

SwissProt

Q4KLP0; Q5XI78; Q6P6R2; Q01205

Entrez Gene

290566; 360975; 298942



  Copyright 2009, Center for Bioinformatics 
  Last Modified: 2009-03-24  
  Design by Zhao Min