BCKDHB Products

Antibodies
BCKDHB Antibody
BCKDHB Antibody
NBP1-86326
Species: Hu, Mu, Rt
Applications: WB, IHC, IHC-P
Host: Rabbit Polyclonal
BCKDHB Antibody
BCKDHB Antibody
NBP2-84500
Species: Hu
Applications: WB
Host: Rabbit Polyclonal
BCKDHB Antibody
BCKDHB Antibody
NBP2-92027
Species: Mu
Applications: WB
Host: Rabbit Polyclonal
Lysates
BCKDHB Overexpression Lysate
BCKDHB Overexpression Lysate
NBL1-07944
Species: Hu
Applications: WB
BCKDHB Overexpression Lysate
BCKDHB Overexpression Lysate
NBL1-07943
Species: Hu
Applications: WB
Proteins
BCKDHB Recombinant Protein An ...
BCKDHB Recombinant Protein Antigen
NBP1-86326PEP
Species: Hu
Applications: AC

Description

Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, andfunctions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components:branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3).This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease(MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physicalretardation, and feeding problems. Alternative splicing at this locus results in transcript variants with different3' non-coding regions, but encoding the same isoform. (provided by RefSeq)

Bioinformatics

Entrez Human
Uniprot Human
Human
Human
Human
Product By Gene ID 594
Alternate Names
  • 2-oxoisovalerate dehydrogenase beta subunit
  • 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
  • BCKDE1B
  • BCKDH E1-beta
  • branched chain alpha-ketoacid dehydrogenase E1-beta subunit
  • branched chain keto acid dehydrogenase E1, beta polypeptide
  • Branched-chain alpha-keto acid dehydrogenase E1 component beta chain
  • dJ279A18.1
  • E1B
  • E1b-beta subunit of the branched-chain complex
  • EC 1.2.4.4
  • FLJ17880

Pathways for BCKDHB

View related products by pathway and learn more about each of the pathways below.

Localization

Bioinformatics Tool for BCKDHB

Discover related pathways, diseases and genes to BCKDHB. Need help? Read the Bioinformatics Tool Guide for instructions on using this tool.
 
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