PDHB polyclonal, anti-human, mouse, rat

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).

Alternative Name:
PDHE1-B, PDHB, PHE1B, DKFZp564K0164, mitochondrial, ODPB, pdhB, PDHBD, PDHE1 B, PDHE1-B, PHE1B, Pyruvate dehydrogenase (lipoamide) beta, Pyruvate dehydrogenase E1 beta polypeptide, Pyruvate dehydrogenase E1 component subunit beta, Pyruvate dehydrogenase E1 component subunit beta mitochondrial,

Application Dilution:
WB:1:500-1:2,000
IHC:1:50-1:200
FC:1:50-1:100

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

Immunogen:
Recombinant protein

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

Purification & Purity:
ProA affinity purified

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

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

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