PDHB Polyclonal Antibody-BS91041

PDHB Polyclonal Antibody

Sizes: 50µl, 100µl

Catalogue Numbers: BS91041-50, BS91041-100

Product: Rabbit IgG, 1mg/ml in PBS with 0.02% sodium azide, 50% glycerol, pH7.2

Swiss-Prot: P11177(Human) Q9D051(Mouse) P49432(Rat)

Host: Rabbit

Reactivity: Human, Mouse, Rat

Applications: WB, IHC, IP, FC

All Applications: WB:1:500-1:2,000
IHC:1:50-1:200
FC:1:50-1:100

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).

Purification and Purity: ProA affinity purified

Storage and Stability: Store at +4°C after thawing. Aliquot store at -20°C or -80°C. Avoid repeated freeze / thaw cycles.

Specificity: PDHB polyclonal antibody detects endogenous levels of PDHB protein.

Immunogen: Recombinant protein

Conjugate: Unconjugated

Modification: Unmodified

Note: For research use only, not for use in diagnostic procedure.

Extra Notes: 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., 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.