DBT, dihydrolipoamide branched chain transacylase E2

DBT, dihydrolipoamide branched chain transacylase E2

• 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]

• Gene Synonyms (lipoamide acyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, mitochondrial, 52 kDa mitochondrial autoantigen of primary biliary cirrhosis, BCKAD E2 subunit, E2 component of branched chain alpha-keto acid dehydrogenase complex, branched chain 2-oxo-acid dehydrogenase complex component E2, branched chain acyltransferase, E2 component, branched-chain alpha-keto acid dehydrogenase complex component E2, dihydrolipoamide acetyltransferase component of branched-chain alpha-keto acid dehydrogenase complex, dihydrolipoyl transacylase, dihydrolipoyllysine-residue (2-methylpropanoyl)transferase, lipoamide acyltransferase component of mitochondrial branched-chain alpha-keto acid dehydrogenase complex, mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit (E2b), BCATE2, BCKAD-E2, BCKADE2, BCOADC-E2, E2, E2B,)
• NCBI Gene ID: 1629
• Species: Homo sapiens (Human)
• UNIPROT ID#>>P11182
• View the NCBI Database for this Gene »

Gene products are often involved in multiple pathways and networks within a living cell. Learn more about other interacting partners.

dihydrolipoamide branched chain transacylase E2 interacts with:

• acireductone dioxygenase 1
  acyl-CoA dehydrogenase family member 9
  argonaute RISC catalytic component 3
  argonaute RISC component 4
  ataxin 3
  ATP binding cassette subfamily C member 1
  autophagy related 16 like 1
  bromodomain containing 7
  carbonic anhydrase 5B
  centriolin
  centrosomal protein 128
  centrosomal protein 152
  centrosomal protein 164
  DDB1 and CUL4 associated factor 11
  DDB1 and CUL4 associated factor 15
  dihydrolipoamide dehydrogenase
  dihydrolipoamide S-succinyltransferase
  double PHD fingers 2
  dynein cytoplasmic 1 intermediate chain 1
  dynein light chain roadblock-type 1
  dynein light chain Tctex-type 1
  enoyl-CoA hydratase, short chain 1
  estrogen receptor 2
  fumarylacetoacetate hydrolase domain containing 2A
  golgi brefeldin A resistant guanine nucleotide exchange factor 1
  GrpE like 2, mitochondrial
  HECT domain E3 ubiquitin protein ligase 1
  HECT, UBA and WWE domain containing E3 ubiquitin protein ligase 1
  histone deacetylase 6
  histone deacetylase 6
  hook microtubule tethering protein 1
  interferon induced protein with tetratricopeptide repeats 2
  isocitrate dehydrogenase (NAD(+)) 3 non-catalytic subunit beta
  lipoyltransferase 1
  mediator complex subunit 4
  mitochondrial ribosomal protein S11
  mitochondrial ribosome recycling factor
  mitochondrial rRNA methyltransferase 1
  MKS transition zone complex subunit 1
  myosin VI
  nephrocystin 1
  neurofibromin 2
  ninein
  nocturnin
  nuclear receptor subfamily 2 group C member 2
  nuclear receptor subfamily 3 group C member 1
  palmitoyl-protein thioesterase 1
  parkin RBR E3 ubiquitin protein ligase
  phosphatase and tensin homolog
  protein kinase D2
  protein phosphatase 6 catalytic subunit
  protein phosphatase targeting COQ7
  protein phosphatase, Mg2+/Mn2+ dependent 1K
  RAS like proto-oncogene B
  RecQ like helicase 4
  RPGRIP1 like
  serine/arginine repetitive matrix 2
  small nuclear ribonucleoprotein U1 subunit 70
  thioesterase superfamily member 4
  thyroid hormone receptor interactor 4
  tRNA methyltransferase 61B
  ubiquitin specific peptidase 16
  zinc binding alcohol dehydrogenase domain containing 2

Paste a protein or nucleic acid sequence in the box below to confirm that it matches this gene’s reference sequence(s). Click on a link under RELATED ORF CLONES to see how a sequence matches to an experimentally-validated ORF clone.