DBT

Entrez Summary: The branched-chain alpha-keto acid dehydrogenase complex (BCKD) is an inner-mitochondrial enzyme complex involved in the breakdown of the branched-chain amino acids isoleucine, leucine, and valine. The BCKD complex is thought to be composed of a core of 24 transacylase (E2) subunits, and associated decarboxylase (E1), dehydrogenase (E3), and regulatory subunits. This gene encodes the transacylase (E2) subunit. Mutations in this gene result in maple syrup urine disease, type 2. Alternatively spliced transcript variants have been described, but their biological validity has not been determined. [provided by RefSeq, Jul 2008].
UniProt Summary: The branched-chain alpha-keto dehydrogenase complex catalyzes the overall conversion of alpha-keto acids to acyl-CoA and CO(2). It contains multiple copies of three enzymatic components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). Within this complex, the catalytic function of this enzyme is to accept, and to transfer to coenzyme A, acyl groups that are generated by the branched-chain alpha-keto acid decarboxylase component.

Pfam Domains GO Terms

Compound Hit Most Correlated Genes in Chemogenomics Tissues where Essential in the Avana Dataset (DepMap 20Q1)

Screen	Score
Alpha-Amanitin 0.5μM R00 exp5	-1.77
∆-9-Tetrahydrocannabinol 30μM R08 exp445	1.77
Artemisinin 50μM R08 exp450	1.91
Torin1 0.08μM R02 exp82	1.99
Aphidicolin 0.4μM R08 exp440	2.34

Global Fraction of Cell Lines Where Essential: 1/739

Tissue	Fraction Of Cell Lines Where Essential
1290807.0	0/1
909776.0	0/1
bile duct	0/28
blood	0/28
bone	0/26
breast	0/33
central nervous system	0/56
cervix	0/4
colorectal	0/17
esophagus	0/13
fibroblast	0/1
gastric	0/16
kidney	0/21
liver	0/20
lung	0/75
lymphocyte	0/16
ovary	0/26
pancreas	0/24
peripheral nervous system	0/16
plasma cell	0/15
prostate	0/1
skin	0/24
soft tissue	0/9
thyroid	0/2
upper aerodigestive	0/22
urinary tract	0/29
uterus	0/5

Expression Distribution

lipoic acid binding
dihydrolipoyllysine-residue (2-methylpropanoyl)transferase activity
mitochondrial alpha-ketoglutarate dehydrogenase complex
acetyltransferase activity
branched-chain amino acid catabolic process
branched-chain amino acid metabolic process
mitochondrial nucleoid
cellular amino acid catabolic process
carboxylic acid catabolic process
organic acid catabolic process
ubiquitin protein ligase binding
cellular amino acid metabolic process
mitochondrial matrix
small molecule catabolic process
carboxylic acid metabolic process
oxoacid metabolic process
organic acid metabolic process
organonitrogen compound catabolic process
mitochondrion
small molecule metabolic process
organic substance catabolic process
cellular catabolic process