Recombinant Rabbit monoclonal primary
Recombinant Pyruvate Dehydrogenase E1 beta subunit Monoclonal Antibody (ET1705-57)
HepG2, Hela, PC-12, mouse kidney, rat heart tissue, human kidney tissue, human stomach cancer tissue, mouse hippocampus tissue, mouse brain tissue.
Store at +4C after thawing. Aliquot store at -20C or -80C. Avoid repeated freeze / thaw cycles.
1*TBS (pH7.4), 0.05% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.
Protein A purified.
Pyruvate Dehydrogenase E1 beta subunit
DKFZp564K0164 antibody; mitochondrial antibody; ODPB_HUMAN antibody; pdhB antibody; PDHBD antibody; PDHE1 B antibody; PDHE1-B antibody; PHE1B antibody; Pyruvate dehydrogenase (lipoamide) beta antibody; Pyruvate dehydrogenase E1 beta polypeptide antibody; Pyruvate dehydrogenase E1 component subunit beta antibody; Pyruvate dehydrogenase E1 component subunit beta mitochondrial antibody
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).