Pyruvate Dehydrogenase E1 beta subunit Recombinant Rabbit mAb IgG
Fig1: Western blot analysis of Pyruvate Dehydrogenase E1 beta subunit on HepG2 (1) and Hela (2) cell lysate using anti-Pyruvate Dehydrogenase E1 beta subunit antibody at 1/1,000 dilution.
Fig2: Western blot analysis of Pyruvate Dehydrogenase E1 beta subunit on mouse kidney tissue (1) and PC-12 (2) cell lysate using anti-Pyruvate Dehydrogenase E1 beta subunit antibody at 1/1,000 dilution.
Fig3: Immunohistochemical analysis of paraffin-embedded rat heart tissue using anti-Pyruvate Dehydrogenase E1 beta subunit antibody. Counter stained with hematoxylin.
Host Species; Species ReactivityRabbit; Human, Mouse, Rat
Application SummaryWB, 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 beta, mitochondrial
BackgroundThe 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).(ET1705-57)