DBT Products

Antibodies
DBT Antibody
DBT Antibody
NBP1-89522
Species: Hu, Mu, Rt
Applications: WB, ICC/IF, IHC, IHC-P
Host: Rabbit Polyclonal
DBT Antibody
DBT Antibody
NBP1-31760
Species: Hu, Mu, Rt
Applications: WB, ICC/IF, IHC, IHC-P
Host: Rabbit Polyclonal
DBT Antibody
DBT Antibody
NBP1-85964
Species: Hu, Mouse, Rat
Applications: WB, IHC, IHC-P
Host: Rabbit Polyclonal
Lysates
DBT Overexpression Lysate (De ...
DBT Overexpression Lysate (Denatured)
H00001629-T01
Species: Hu
Applications: WB
DBT Overexpression Lysate (Na ...
DBT Overexpression Lysate (Native)
NBL1-09735
Applications: WB
Proteins
DBT Recombinant Protein Antig ...
DBT Recombinant Protein Antigen
NBP1-85963PEP
Species: Hu
Applications: AC
DBT Recombinant Protein Antig ...
DBT Recombinant Protein Antigen
NBP1-85964PEP
Species: Hu
Applications: AC
DBT Recombinant Protein Antig ...
DBT Recombinant Protein Antigen
NBP1-89522PEP
Species: Hu
Applications: AC

Description

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]

Bioinformatics

Entrez Human
Uniprot Human
Human
Human
Product By Gene ID 1629
Alternate Names
  • Dihydrolipoamide branched chain transacylase
  • E2B
  • EC 2.3.1.168
  • E2
  • mitochondrial branched chain alpha-keto acid dehydrogenase transacylase subunit(E2b)
  • dihydrolipoamide branched chain transacylase (E2 component of branched chainketo acid dehydrogenase complex; maple syrup urine disease)
  • lipoamide acyltransferase component of mitochondrial branched-chain alpha-ketoacid dehydrogenase complex
  • BCKADE2
  • lipoamide acyltransferase component of branched-chain alpha-keto aciddehydrogenase complex, mitochondrial
  • BCKAD-E2
  • E2 component of branched chain alpha-keto acid dehydrogenase complex
  • Dihydrolipoyllysine-residue (2-methylpropanoyl)transferase
  • dihydrolipoyl transacylase
  • Dihydrolipoamide acetyltransferase component of branched-chain alpha-keto aciddehydrogenase complex
  • branched chain acyltransferase, E2 component
  • Branched-chain alpha-keto acid dehydrogenase complex component E2
  • BCATE2BCKAD E2 subunit
  • MGC9061
  • dihydrolipoamide branched chain transacylase E2

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