Q658P7 · Q658P7_HUMAN

  • Protein
    2-oxoisovalerate dehydrogenase subunit alpha
  • Gene
    DKFZp313C0640
  • Status
    UniProtKB unreviewed (TrEMBL)
  • Amino acids
  • Protein existence
    Evidence at transcript level
  • 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

Gene names

    • Name
      DKFZp313C0640

Organism names

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

Accessions

  • Primary accession
    Q658P7

Subcellular Location

PTM/Processing

Proteomic databases

Family & Domains

Features

Showing features for region, compositional bias, domain.

TypeIDPosition(s)Description
Region33-52Disordered
Compositional bias37-51Polar residues
Domain106-319Dehydrogenase E1 component

Sequence similarities

Belongs to the BCKDHA family.

Keywords

Family and domain databases

Sequence

  • Sequence status
    Complete
  • Length
    403
  • Mass (Da)
    44,353
  • Last updated
    2004-10-25 v1
  • Checksum
    A8CEB2C9C6741D2E
MAVAIAAARVWRLNRGLSQAALLLLRQPGARGLARSHPPRQQQQFSSLDDKPQFPGASAEFIDKLEFIQPNVISGIPIYRVMDRQGQIINPSEDPHLPKEKVLKLYKSMTLLNTMDRILYESQRQGRISFYMTNYGEEGTHVGSAAALDNTDLVFGQYREAGVLMYRDYPLELFMAQCYGNISDLGKGRQMPVHYGCKERHFVTISSPLATQIPQAVGAAYAAKRANANRVVICYFGEGAASEGDAHAGFNFAATLECPIIFFCRNNGYAISTPTSEQYRGDGIAARGPGYGIMSIRVDGNDVFAVYNATGGPKARASVGPSSPSTTGTCGIMMPLHAMGLWHPVSHQQELSTAPKPKGTTKPILESYLKSHLLGHSRYQLYIHQDAVRREKPQSNLNRESLI

Features

Showing features for compositional bias.

TypeIDPosition(s)Description
Compositional bias37-51Polar residues

Sequence databases

Nucleotide SequenceProtein SequenceMolecule TypeStatus
AL833143
EMBL· GenBank· DDBJ
CAH56215.1
EMBL· GenBank· DDBJ
mRNA

Similar Proteins

Disclaimer

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