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

Basic Information

Enzyme Number

Official Name

dihydrolipoyl dehydrogenase

Name from literature

branched-chain alpha-keto acid dehydrogenase complex

Pathway from literature

the branched-chain amino acid catabolism

Pathway from KEGG

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

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

Carbohydrate Metabolism; Pyruvate metabolism; map00620

Carbohydrate Metabolism; Glycolysis / Gluconeogenesis; map00010

Carbohydrate Metabolism; Citrate cycle (TCA cycle); map00020

Amino Acid Metabolism; Alanine and aspartate metabolism; map00252


Mouse (10090)

Genome localization

12 A3|12 15.1 cM[13382 ],


A flavoprotein (FAD). A component of the multienzyme 2-oxo-acid dehydrogenase complexes. In the pyruvate dehydrogenase complex, it binds to the core of EC, dihydrolipoyllysine-residue acetyltransferase, and catalyses oxidation of its dihydrolipoyl groups. It plays a similar role in the oxoglutarate and 3-methyl-2-oxobutanoate dehydrogenase complexes. Another substrate is the dihydrolipoyl group in the H-protein of the glycine-cleavage system (click here for diagram), in which it acts, together with EC, glycine dehydrogenase (decarboxylating), and EC, aminomethyltransferase, to break down glycine. It can also use free dihydrolipoate, dihydrolipoamide or dihydrolipoyllysine as substrate. This enzyme was first shown to catalyse the oxidation of NADH by methylene blue; this activity was called diaphorase. The glycine cleavage system is composed of four components that only loosely associate: the P protein (EC, the T protein (EC, the L protein (EC and the lipoyl-bearing H protein [6].

Rate-limiting Description

"The BCKDH (branched-chain alpha-keto acid dehydrogenase complex) catalyses the rate-limiting step in the oxidation of BCAAs (branched-chain amino acids)." (16875466)

Gene ontology

Gene ontology

GO:0050660 (F) FAD binding [Q3TIE8, O08749 ];
GO:0006508 (P) proteolysis [O08749 ];
GO:0048240 (P) sperm capacitation [O08749 ];
GO:0005737 (C) cytoplasm [Q3TIE8 ];
GO:0006120 (P) mitochondrial electron transport, NADH to u... [O08749 ];
GO:0019861 (C) flagellum [O08749 ];
GO:0004148 (F) dihydrolipoyl dehydrogenase activity [Q3TIE8, O08749 ];
GO:0007369 (P) gastrulation [O08749 ];
GO:0005759 (C) mitochondrial matrix [O08749 ];
GO:0009055 (F) electron carrier activity [Q3TIE8, O08749 ];
GO:0043159 (C) acrosomal matrix [O08749 ];
GO:0055114 (P) oxidation reduction [Q3TIE8 ];
GO:0045454 (P) cell redox homeostasis [Q3TIE8, O08749 ];
GO:0042391 (P) regulation of membrane potential [O08749 ];

Subcellular localization


mitochondrion [O08749 ];



O08749; Q3TIE8

Entrez Gene


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