PDHB polyclonal Antibody | BS91041

PDHB polyclonal Antibody | BS91041 | Gentaur UK, US & Europe Distribution

Host: Rabbit

Reactivity: Human, Mouse, Rat

Application: WB IHC IP FC

Application Range: 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) .

Storage & 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.

Molecular Weight: N/A

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

Alternative Names: 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,

Immunogen: Recombinant protein

Conjugate: Unconjugated

Modification: Unmodification

Purification & Purity: ProA affinity purified

Pathway: