Product Datasheet  
BCKDHB Polyclonal Antibody  
Catalog Number: 27467  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P21953
    NCBI Gene ID:594
  • Form of Antibody：  
  • Avoid freeze / thaw cycles.|Buffer: PBS with 50% glycerol, pH7.4.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant fusion protein of human BCKDHB (NP_898871.1).
  • reactivity：  
  • Mouse
  • appl_detail：  
  • WB 1:500 - 1:2000
  • other_names：  
  • BCKDHB; BCKDE1B; BCKDH E1-beta; E1B; BCKDHE1-beta; branched chain keto acid dehydrogenase E1 subunit beta
  • Purification：  
  • Affinity purification
  • Specificity：  
  • Applications：  
  • WB
  • Background：  
  • 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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