Q9UMB3 · Q9UMB3_HUMAN
- Protein2-oxoisovalerate dehydrogenase subunit alpha
- StatusUniProtKB unreviewed (TrEMBL)
- Organism
- Amino acids
- Protein existenceInferred from homology
- Annotation score2/5
Function
function
Together with BCKDHB forms the heterotetrameric E1 subunit of the mitochondrial branched-chain alpha-ketoacid dehydrogenase (BCKD) complex. The BCKD complex catalyzes the multi-step oxidative decarboxylation of alpha-ketoacids derived from the branched-chain amino-acids valine, leucine and isoleucine producing CO2 and acyl-CoA which is subsequently utilized to produce energy. The E1 subunit catalyzes the first step with the decarboxylation of the alpha-ketoacid forming an enzyme-product intermediate. A reductive acylation mediated by the lipoylamide cofactor of E2 extracts the acyl group from the E1 active site for the next step of the reaction.
Catalytic activity
- 3-methyl-2-oxobutanoate + H+ + N6-[(R)-lipoyl]-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase] = CO2 + N6-[(R)-S8-2-methylpropanoyldihydrolipoyl]-L-lysyl-[dihydrolipoyllysine-residue (2-methylpropanoyl)transferase]
Cofactor
GO annotations
Aspect | Term | |
---|---|---|
Molecular Function | 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity | |
Biological Process | branched-chain amino acid catabolic process |
Keywords
- Molecular function
- Ligand
Enzyme and pathway databases
Names & Taxonomy
Protein names
- Recommended name2-oxoisovalerate dehydrogenase subunit alpha
- EC number
- Alternative names
Organism names
- Organism
- Taxonomic lineageEukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo
Accessions
- Primary accessionQ9UMB3
PTM/Processing
Proteomic databases
Structure
Sequence
- Sequence statusFragment
- Length56
- Mass (Da)6,701
- Last updated2000-05-01 v1
- Checksum651EA30E73C39C94
Features
Showing features for non-terminal residue.
Type | ID | Position(s) | Description | |||
---|---|---|---|---|---|---|
Non-terminal residue | 1 | |||||
Sequence: V |