Q9UMB3 · Q9UMB3_HUMAN

  • Protein
    2-oxoisovalerate dehydrogenase subunit alpha
  • Status
    UniProtKB unreviewed (TrEMBL)
  • Amino acids
  • Protein existence
    Inferred from homology
  • Annotation score
    2/5

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

Cofactor

thiamine diphosphate (UniProtKB | Rhea| CHEBI:58937 )

Keywords

Enzyme and pathway databases

Names & Taxonomy

Protein names

  • Recommended name
    2-oxoisovalerate dehydrogenase subunit alpha
  • EC number
  • Alternative names
    • Branched-chain alpha-keto acid dehydrogenase E1 component alpha chain

Organism names

  • Taxonomic identifier
  • Organism
  • Taxonomic lineage
    Eukaryota > Metazoa > Chordata > Craniata > Vertebrata > Euteleostomi > Mammalia > Eutheria > Euarchontoglires > Primates > Haplorrhini > Catarrhini > Hominidae > Homo

Accessions

  • Primary accession
    Q9UMB3

PTM/Processing

Proteomic databases

Family & Domains

Sequence similarities

Belongs to the BCKDHA family.

Family and domain databases

Sequence

  • Sequence status
    Fragment
  • Length
    56
  • Mass (Da)
    6,701
  • Last updated
    2000-05-01 v1
  • Checksum
    651EA30E73C39C94
VMEAFEQAERKPKPNPNLLFSDVYQEMPAQLRKQQESLARHLQTYGEHYPLDHFDK

Features

Showing features for non-terminal residue.

TypeIDPosition(s)Description
Non-terminal residue1

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
S38309
EMBL· GenBank· DDBJ
AAB19267.1
EMBL· GenBank· DDBJ
Genomic DNA

Similar Proteins

Disclaimer

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