Anti-BCKDHB antibody

Catalog Number:

A001020299STJ

Mfr. No.:

STJ112553

Price:

$297

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Overview
- This gene encodes the E1 beta subunit of branched-chain keto acid dehydrogenase, which is a multienzyme complex associated with the inner membrane of mitochondria. This enzyme complex functions in the catabolism of branched-chain amino acids. Mutations in this gene 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 physical retardation and feeding problems. Alternative splicing at this locus results in multiple transcript variants.
  
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Properties
- Host
  
  rabbit
  
  Antibody Type
  
  polyclonal
  
  Isotype
  
  IgG
  
  Reactivity
  
  mouse
  
  Immunogen
  
  Recombinant fusion protein containing a sequence corresponding to amino acids 183-392 of human BCKDHB (NP_898871. 1).
  
  Purification
  
  Affinity purification
  
  Conjugate
  
  Unconjugated
  
  Formulation
  
  PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
  
  Storage
  
  Store at -20°C and avoid freeze-thaw cycles.
  
  Regulatory Status
  
  RUO
  
  Antigen
  
  2-Oxoisovalerate Dehydrogenase Subunit Beta-Mitochondrial; Branched-Chain Alpha-Keto Acid Dehydrogenase E1 Component Beta Chain; Bckde1b; Bckdh E1-Beta (Gene ID:594; UniProt:P21953)
  
  * For research use only