BT-MCA4517

Mouse

Mouse IgG1

100μL,50μL

Monoclonal

Purified recombinant fragment of human PDHA1(AA: 241-390) expressed in E. Coli.

Purified antibody in PBS with 0.05% sodium azide

Store at 4°C short term. Aliquot and store at -20°C long term.

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.

P08559:;

43 kDa

5160

PDHA;PDHAD;PHE1A;PDHCE1A

Signal Transduction,Metabolism

PDHA1