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

Basic Information

Enzyme Number

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


Human (9606)

Genome localization

10q11.23[55753 ], 7p14-p13[4967 ], 14q24.3[1743 ], 7q31-q32[1738 ],


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

Rate-limiting Description

"The enzymes that form the mitochondrial alpha-ketoglutarate- dehydrogenase complex (KGDHC), a key and arguably rate-limiting enzyme system of the tricarboxylic acid cycle, might mediate the interaction of these processes." (15953811)

"The earliest metabolic consequence of thiamine deficiency is a selective loss in activity of the thiamine diphosphate-dependent enzyme alpha-ketoglutarate dehydrogenase (alpha-KGDH), a rate-limiting tricarboxylic acid cycle enzyme." (15953809)

Regulatory Information

Upstream transcription factor


Regulatory type


key enzyme;

"A novel regulatory mechanism for control of the ubiquitous 2-oxoglutarate dehydrogenase complex (ODH), a key enzyme of the tricarboxylic acid cycle, was discovered in the actinomycete Corynebacterium glutamicum, a close relative of important human pathogens like Corynebacterium diphtheriae and Mycobacterium tuberculosis." (16522631)


"Corynebacterial protein kinase G controls 2-oxoglutarate dehydrogenase activity via the phosphorylation status of the OdhI protein." (16522631)

transcriptional factor;

"Functional characterization of the 5'-flanking region of the gene encoding human 2-oxoglutarate dehydrogenase.We have identified a nuclear factor that binds to nt -63 to -24 including two cis-acting sites." (9047343)

transcriptional factor;

"Only 33% of the functional variants were found in known consensus transcription factor binding sequences or motifs, which suggests that either there are many unknown transcription factor binding motifs or other, unknown mechanisms are involved. The genes with functional polymorphisms that are reported here for the first time include AGTRL2, CAT, CHRNA5, CTSG, CYP2D6, DLD, ERCC1, GABRA1, GABRP, HNRPH3, HIP1, IGKV1-9, KCNJ15, KCNK6, KLK1, MSMB, MYOC, NPY2R, NOTCH4, ORM2, PEDF, PTPRCAP, ST16 (IL24), SULT1A1, and TSHR." (16086313)

interact with TF;ZHX1(11244)

"A human protein-protein interaction network: a resource for annotating the proteome." (16169070)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [P09622 ];
GO:0006096 (P) glycolysis [Q96HY7, Q02218 ];
GO:0045252 (C) oxoglutarate dehydrogenase complex [P36957 ];
GO:0004148 (F) dihydrolipoyl dehydrogenase activity [P09622 ];
GO:0031405 (F) lipoic acid binding [P36957 ];
GO:0006099 (P) tricarboxylic acid cycle [P36957 ];
GO:0009055 (F) electron carrier activity [P09622 ];
GO:0005515 (F) protein binding [P36957 ];
GO:0045454 (P) cell redox homeostasis [P09622 ];
GO:0004591 (F) oxoglutarate dehydrogenase (succinyl-transf... [Q96HY7, Q02218 ];
GO:0030976 (F) thiamin pyrophosphate binding [Q96HY7, Q02218 ];
GO:0031966 (C) mitochondrial membrane [Q02218 ];
GO:0005739 (C) mitochondrion [Q96HY7 ];
GO:0005759 (C) mitochondrial matrix [P09622, P36957, Q02218 ];
GO:0005634 (C) nucleus [P36957 ];
GO:0055114 (P) oxidation reduction [P09622, Q96HY7, Q02218 ];
GO:0004149 (F) dihydrolipoyllysine-residue succinyltransfe... [P36957 ];

Subcellular localization


mitochondrion [P09622, P36957, Q96HY7, Q02218 ];

Disease relevance


Defects in DLD are a cause of congenital infantile lactic acidosis [P09622 ];

Defects in DLD are a cause of maple syrup urine disease (MSUD) [MIM:248600]. MSUD is characterized by mental and physical retardation, feeding problems and a maple syrup odor to the urine. The keto acids of the branched-chain amino acids are present in the urine, resulting from a block in oxidative decarboxylation [P09622 ];



Q02218; Q96HY7; P09622; P36957

Entrez Gene

4967; 55753; 1738; 1743


02006; 08938; 00520; 07704

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