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©2020 by Bon Opus Biosciences, LLC.

PDHA1 Recombinant Rabbit monoclonal Antibody IgG

SKU: BA112078-100µl
$279.00Price

Fig1: Western blot analysis of PDHA1 on different lysates using anti-PDHA1 antibody at 1/1,000 dilution.

Positive control:   

Lane 1: 293T            

Lane 2: A431 

Lane 3: Mouse heart

Fig2: ICC staining PDHA1 in Hela cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Fig3: ICC staining PDHA1 in HepG2 cells (red). The nuclear counter stain is DAPI (blue). Cells were fixed in paraformaldehyde, permeabilised with 0.25% Triton X100/PBS.

Bon Opus Cat. #BA112078
Size
  • Host Species; Species Reactivity

    Rabbit; Human, Mouse, Rat
  • Immunogen

    Recombinant protein
  • Application Summary

    WB, ICC/IF, IHC, IP, FC
  • Purification; Formulation

    ProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
  • ALTnames

    Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial, PDHE1-A type I
  • Background

    The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The E1 enzyme of the PDH complex is made up of a heterotetramer of two α and two β subunits. The E1-α subunit (PDH-E1α) contains the E1 active site and plays a key role in the function of the PDH complex. The PDH complex is regulated by phosphorylation and dephosphorylation of PDH-E1α. The gene encoding for PDH-E1α maps to chromosome Xp22.12, and a 20bp deletion in the last exon of this gene is sufficient to cause PDH deficiency, which causes a broad range of symptoms including the development of seizures, mental retardation and spasticity, as well as intermittent episodes of lactic acidosis associated with cerebellar ataxia.(ET1702-75)