Product Name | Pyruvate Dehydrogenase E1 alpha Rabbit mAb |
Background | 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.[provided by RefSeq, Mar 2010] |
Reactivity | Human; Mouse; Rat |
Source | Rabbit, IgG Kappa |
Applications | WB; IHC; IF; IP; ELISA |
Dilutions | WB: 1:1000-1:5000; IHC: 1:200-1:1000; IF: 1:200-1:1000; IP: 1:50-1:200; ELISA: 1:5000-1:20000 |
Purification | Protein A |
Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
Storage and Stability | -20°C/1 year |
MW (kDa) | Calculated: 43; Observed: 43 |
UniProt Accession # | P08559 |
Protein Name | Pyruvate Dehydrogenase E1 alpha |
Gene Name | PDHA1 |
Gene ID | 5160 |
Other Names | PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I |