PDHA1 Polyclonal Antibody

Optimal working dilutions should be determined experimentally by the investigator. Suggested starting dilutions are as follows: WB (1:500-1:2000), IHC-P (1:100-1:300), ELISA (1:40000). Not yet tested in other applications.

Liquid solution

PBS containing 50% Glycerol, 0.5% BSA and 0.02% Sodium Azide.

Gel pack with blue ice.

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. PDHA1 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 PDHA1 are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for PDHA1.

PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I

P08559