PDHA1 Antibody

Catalog Number:

49389

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P08559

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, ICC/IF, IHC, IP, FC

Background:

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial matrix enzyme complex that functions as the primary link between glycolysis and the tricarboxylic acid (TCA) cycle by catalyzing the irreversible conversion of pyruvate into acetyl-CoA. The E1 enzyme of the PDH complex is made up of a heterotetramer of two α and two β subunits. The E1-α subunit (PDH-E1α) contains the E1 active site and plays a key role in the function of the PDH complex. The PDH complex is regulated by phosphorylation and dephosphorylation of PDH-E1α. The gene encoding for PDH-E1α maps to chromosome Xp22.12, and a 20bp deletion in the last exon of this gene is sufficient to cause PDH deficiency, which causes a broad range of symptoms including the development of seizures, mental retardation and spasticity, as well as intermittent episodes of lactic acidosis associated with cerebellar ataxia.

References:

appl_detail:

WB: 1:1,000-5,000
IHC: 1:50-1:200
ICC: 1:50-1:200

FC: 1:50-1:100

Datasheet PDF