top of page

PDHA1 mAb

The pyruvate dehydrogenase complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO, and provides the primary link between glycolysis and the tricarboxylic acid cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase , dihydrolipoamide acetyltransferase and lipoamide dehydrogenase. 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.

Alternative Name(s)

Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrialmitochondrial; PDHA1; pdha1a; pdha1b; PHE1A; pyruvate dehydrogenase (lipoamide) alpha 1; pyruvate dehydrogenase E1 alpha 1; Pyruvate dehydrogenase E1 component subunit alpha; somatic form

Application

WB

Read More >

UNIprot

P08559

clonality

Monoclonal

reactivity

Human,Mouse,Rat

Research area

Signal Transduction; Metabolism

source

Mouse

storage

Store at -20℃, stable for one year from the date of shipment.

PDHA1 mAb

bottom of page