名称：PDHA1

别名：PDHA; PDHAD; PHE1A; PDHCE1A

Targets：Disease

描述

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.

Specification

Swissprot：P08559

Gene Accession：BC002406

WB Predicted band size：43 kDa

Host/Isotype：Rabbit IgG

Antibody Type：Primary antibody

Storage：Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.

Species Reactivity：Human, Mouse, Rat

免疫原：Fusion protein of human PDHA1

Formulation：Purified antibody in PBS with 0.05% sodium azide and 50% glycerol.

Application

Application：WB,IHC

WB：1/1000-1/5000

IHC：1/50-1/300

ELISA：1/5000-1/10000

宿主：Rabbit