You are using an outdated browser. Please upgrade your browser to improve your experience.

Tbio
BCKDHA
2-oxoisovalerate dehydrogenase subunit alpha, 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). The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by ...more
Uniprot Accession IDs
Gene Name
Ensembl ID
  • ENST00000269980
  • ENSP00000269980
  • ENSG00000248098
  • ENST00000457836
  • ENSP00000416000

Symbol
  • MSU
  • MSUD1
  • OVD1A
  • BCKDE1A
Illumination Graph
Knowledge Table
Most Knowledge About
Knowledge Value (0 to 1 scale)
histone modification site profile
0.91
disease perturbation
0.87
drug perturbation
0.87
interacting protein
0.78
cellular component
0.7


Related Tools
Target Illumination GWAS Analytics (TIGA)
TIGA scores and ranks GWAS discovered associations according to the quantity and quality of the evidence supporting the association.
GENEVA
GENEVA (GENe Expression Variance Analysis) allows you to identify RNA-sequencing datasets from the Gene Expression Omnibus (GEO) that contain conditions modulating a gene or a gene signature.
GlyGen
GlyGen is a data integration and dissemination project for carbohydrate and glycoconjugate related data.
ARCHS4
ARCHS4 provides access to gene-function predictions based on RNA-seq co-expression, and gene expression levels across cell and tissues.
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: 55.96   (req: < 5)
Gene RIFs: 22   (req: <= 3)
Antibodies: 115   (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: 55.96   (req: >= 5)
Gene RIFs: 22   (req: > 3)
Antibodies: 115   (req: > 50)

- OR - satisfy the following criterion:

Gene Ontology Terms: 5
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
GWAS Traits (2)
GWAS Trait
EFO ID
Study Count
SNP Count
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
balding measurement
1
1
1
89.8
body height
2
2
2
84.2
GWAS Trait
EFO ID
Beta Count
Odds Ratio
Evidence (Mean Rank Score)
Provenance
balding measurement
1
89.8
body height
2
84.2
Orthologs (12)
1 – 5 of 12
Species
Name
Source ID
Gene ID
OMA
EggNOG
Inparanoid
Chimp
branched chain keto acid dehydrogenase E1, alpha polypeptide
468889
Macaque
transmembrane protein 91
100426021
Mouse
MGI:107701
12039
Rat
RGD:2196
25244
Horse
branched chain keto acid dehydrogenase E1, alpha polypeptide
100068678
Species
Name
OMA
EggNOG
Inparanoid
Chimp
branched chain keto acid dehydrogenase E1, alpha polypeptide
Macaque
transmembrane protein 91
Mouse
Rat
Horse
branched chain keto acid dehydrogenase E1, alpha polypeptide
Protein Structure (24 Structures, 1 AlphaFold Model)
RepresentationColor Scheme

Click on a row in the table to change the structure displayed.
More information can be found at RCSB PDB

AF-P12694-F1-model_v1

AlphaFold Structures Developed by DeepMind and EMBL-EBI

1 – 5 of 24
PDB Structure Id
Ligand
Method
Resolution (Å)
Residues
Fraction of Total Protein
Pub Year
Title
PDB Structure Id
Fraction of Total Protein
Resolution
Pub Year
Pathways (39)
Branched-chain amino acid catabolism (R-HSA-70895)

Click on a row in the table to change the structure displayed.

Items per page:
1 – 4 of 4
Data Source
Name
Explore in Pharos
Explore in Source
Reactome
Branched-chain amino acid catabolism
Reactome
Glyoxylate metabolism and glycine degradation
Reactome
Metabolism
Reactome
Metabolism of amino acids and derivatives
Name
Explore in Pharos
Explore in Source
Branched-chain amino acid catabolism
Glyoxylate metabolism and glycine degradation
Metabolism
Metabolism of amino acids and derivatives
Protein-Protein Interactions (96)
1 – 10 of 96
PPM1K
Tbio
Family: Enzyme
Novelty: 0.0337866
p_int: 0.993705969
p_ni: 0.00629403
p_wrong: 1e-9
Score: 0.994
Data Source: BioPlex,Reactome,STRINGDB
BCKDHB
Tbio
Family: Enzyme
Novelty: 0.02234969
p_int: 0.980575039
p_ni: 0.019424961
Score: 0.999
Data Source: BioPlex,Reactome,STRINGDB
POLB
Tchem
Family: Enzyme
Novelty: 0.00145102
p_int: 0.91989167
p_ni: 0.08010833
Score: 0.833
Data Source: BioPlex,STRINGDB
S100A4
Tbio
Novelty: 0.00136919
p_int: 0.833787844
p_ni: 0.166212156
Data Source: BioPlex
PDHB
Tbio
Family: Enzyme
Novelty: 0.01018086
Score: 0.997
Data Source: STRINGDB
DBT
Tbio
Family: Enzyme
Novelty: 0.0070589
Score: 0.992
Data Source: Reactome,STRINGDB
DLD
Tbio
Family: Enzyme
Novelty: 0.00214298
Score: 0.989
Data Source: Reactome,STRINGDB
PDHX
Tbio
Family: Enzyme
Novelty: 0.00946631
Score: 0.969
Data Source: STRINGDB
DLAT
Tbio
Family: Enzyme
Novelty: 0.00311191
Score: 0.968
Data Source: STRINGDB
BCKDK
Tbio
Family: Kinase
Novelty: 0.03296998
Score: 0.966
Data Source: Reactome,STRINGDB
Publication Statistics
PubMed Score  55.96

PubMed score by year
PubTator Score  34.56

PubTator score by year
Amino Acid Sequence
Residue Counts
Protein Sequence
ProtVista Viewer
Find similar targets by: