PDHA1 Recombinant Rabbit monoclonal Antibody IgG
Fig1: Western blot analysis of PDHA1 on different lysates using anti-PDHA1 antibody at 1/1,000 dilution.
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.
Host Species; Species ReactivityRabbit; Human, Mouse, Rat
Application SummaryWB, ICC/IF, IHC, IP, FC
Purification; FormulationProA affinity purified; 1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.; Liquid form.
ALTnamesPyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial, PDHE1-A type I
BackgroundThe 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)