Product Datasheet  
Pyruvate Dehydrogenase E1 beta subunit Antibody  
Catalog Number: 49646  
Technical:tech@swbio.com  
Information:info@swbio.com  
Description  
  • host_species：  
  • Rabbit
  • Amount：  
  • 100μgμg
  • Swiss-Prot No.：  
  • Swiss-Prot#:P11177
  • Form of Antibody：  
  • 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
  • Storage：  
  • Store at -20˚C
  • Immunogen：  
  • Recombinant protein
  • reactivity：  
  • Hu, Ms, Rt
  • appl_detail：  
  • WB: 1:500-1:2,000
    IHC: 1:50-1:200


    FC: 1:50-1:100
  • other_names：  
  • DKFZp564K0164 antibody
    mitochondrial antibody
    ODPB_HUMAN antibody
    pdhB antibody
    PDHBD antibody
    PDHE1 B antibody
    PDHE1-B antibody
    PHE1B antibody
    Pyruvate dehydrogenase (lipoamide) beta
  • Purification：  
  • ProA affinity purified
  • Specificity：  
  • Applications：  
  • WB, IHC, IP, FC
  • Background：  
  • The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO. It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency). PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).



 
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