Complex: BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE COMPLEX

General Information

Name

BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE COMPLEX

Species

D. melanogaster

Symbol

BCKDC

FlyBase ID

FBgg0001928

Date last reviewed

2025-04-03

Number of members

Description

The Branched-Chain Alpha-Ketoacid Dehydrogenase Complex is organized around a 24-meric cubic core of the dihydrolipoyl transacylase (E2) with multiple (~6-12) copies of the branched-chain alpha-ketoacid dehydrogenase (E1), ~6 copies of the dimeric E3. The E1 component is a thiamine diphosphate-dependent enzyme consisting of two alpha subunits and two beta subunits. This multienzyme complex functions in the mitochondrial matrix to catalyze the oxidative decarboxylation of branched-chain alpha-ketoacids derived from the branched-chain amino acids leucine, isoleucine and valine. (Adapted from PMID:10745006.)

Notes on Group

The mammalian BCKDC contains an E1-specific kinase and phosphatase (BCKDK and PPM1K, see PMID:29779826), but orthologs or functional homologs of these have not been identified in Drosophila.

The overall reaction catalyzed by the BCKDC is represented by GO:0047101/EC:1.2.1.25, whereas the individual reactions catalyzed by the E1, E2 and E3 components are represented by GO:0003863/EC:1.2.4.4, GO:0043754/EC:2.3.1.168 and GO:0004148/EC:1.8.1.4, respectively.

Source Material

The BRANCHED-CHAIN ALPHA-KETOACID DEHYDROGENASE COMPLEX Gene Group has been compiled using the following publication(s): Marygold, 2024 and Marygold, 2024.1.15 .

Key Gene Ontology (GO) terms

Molecular Function

branched-chain 2-oxo acid dehydrogenase activity

dihydrolipoyl dehydrogenase (NADH) activity

dihydrolipoamide branched chain acyltransferase activity

Biological Process

branched-chain amino acid catabolic process

branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA

Cellular Component

branched-chain alpha-ketoacid dehydrogenase complex

mitochondrial matrix

Enzymatic activity