BCKDHB Products

Antibodies
BCKDHB Antibody
BCKDHB Antibody
NBP2-29984
Species: Hu
Applications: WB, Flow, ICC/IF, IHC-P
Host: Rabbit Polyclonal
BCKDHB Antibody
BCKDHB Antibody
NBP1-86326
Species: Hu, Mouse, Rat
Applications: WB, IHC, IHC-P
Host: Rabbit Polyclonal
Lysates
BCKDHB Overexpression Lysate ...
BCKDHB Overexpression Lysate (Native)
NBL1-07944
Applications: WB
BCKDHB Overexpression Lysate ...
BCKDHB Overexpression Lysate (Native)
NBL1-07943
Applications: WB
Proteins
BCKDHB Recombinant Protein
BCKDHB Recombinant Protein
H00000594-P01
Species: Hu
Applications: WB, ELISA, PA
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
  • E1b-beta subunit of the branched-chain complex
  • E1B
  • dJ279A18.1
  • EC 1.2.4.4
  • branched chain alpha-ketoacid dehydrogenase E1-beta subunit
  • BCKDH E1-beta
  • FLJ17880
  • BCKDE1B
  • 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial
  • Branched-chain alpha-keto acid dehydrogenase E1 component beta chain
  • 2-oxoisovalerate dehydrogenase beta subunit
  • branched chain keto acid dehydrogenase E1, beta polypeptide

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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