Product Datasheet  
BCKDHA Polyclonal Antibody  
Catalog Number: 31741  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P12694
    NCBI Gene ID:593
  • 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 BCKDHA (NP_000700.1).
  • reactivity：  
  • Human,Mouse,Rat
  • appl_detail：  
  • WB 1:500 - 1:2000
  • other_names：  
  • BCKDHA; BCKDE1A; MSU; MSUD1; OVD1A; 2-oxoisovalerate dehydrogenase subunit alpha, mitochondrial
  • Purification：  
  • Affinity purification
  • Specificity：  
  • Applications：  
  • WB
  • Background：  
  • The branched-chain alpha-keto acid (BCAA) dehydrogenase (BCKD) complex is an innter mitochondrial enzyme complex that catalyzes the second major step in the catabolism of the branched-chain amino acids leucine, isoleucine, and valine. The BCKD complex consists of three catalytic components: a heterotetrameric (alpha2-beta2) branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). This gene encodes the alpha subunit of the decarboxylase (E1) component. Mutations in this gene result in maple syrup urine disease, type IA. Multiple transcript variants encoding different isoforms have been found for this gene.



 
© Signalway Biotechnology All Rights Reserved.