Tbio DBT Lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial

Protein Summary

Description

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. 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 Accession IDs

• P11182
• B2R811
• Q5VVL8

Gene Name

• DBT

Ensembl ID

• ENST00000370132
• ENSP00000359151
• ENSG00000137992

Symbol

• BCATE2
• E2
• E2B
• BCATE2
• BCKADE2
• BCKAD-E2
• BCOADC-E2

Knowledge Table

Most Knowledge About	Knowledge Value (0 to 1 scale)
co-expressed gene	0.96
gene perturbation	0.96
protein domain	0.92
histone modification site profile	0.91
protein complex	0.83

Protein Classes

PANTHER Classes

Protein / Transferase / Acyltransferase / Lipoamide Acyltransferase Component Of Branched-chain Alpha-keto Acid Dehydrogenase Complex, Mitochondrial

Protein / Transferase / Acetyltransferase / Lipoamide Acyltransferase Component Of Branched-chain Alpha-keto Acid Dehydrogenase Complex, Mitochondrial

DTO Classes

Protein / Enzyme / Transferase / Acetyltransferase / Lipoamide Acyltransferase Component Of Branched-chain Alpha-keto Acid Dehydrogenase Complex, Mitochondrial

IDG Development Level Summary

Tdark

These are targets about which virtually nothing is known. They do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 192.68   (req: < 5)

Gene RIFs: 15   (req: <= 3)

Antibodies: 175   (req: <= 50)

Tbio

These targets do not have known drug or small molecule activities
- AND - satisfy two or more of the following criteria:

Pubmed score: 192.68   (req: >= 5)

Gene RIFs: 15   (req: > 3)

Antibodies: 175   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 4

Tchem

Target has at least one ChEMBL compound with an activity cutoff of < 30 nM - AND - satisfies the preceding conditions

Active Ligand: 0

Tclin

Target has at least one approved drug - AND - satisfies the preceding conditions

Active Drug: 0