Pyruvate Dehydrogenase E1 beta subunit Antibody

Catalog Number:

49646

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P11177

Form of Antibody:

1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.

Storage/Stability:

Immunogen:

Recombinant protein

Purification:

ProA affinity purified

Specificity/Sensitivity:

Applications:

WB, IHC, IP, FC

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).

References:

appl_detail:

WB: 1:500-1:2,000
IHC: 1:50-1:200


FC: 1:50-1:100

Datasheet PDF