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| Cat No | BMA-1109 |
| Conjugate | |
| Type | 重组兔多抗 |
| Source | Rabbit |
| Size | 50 uL |
| Application | Immunogen |
| Format | Liquid |
| Concentration | Please refer to the vial lable for the specific concentration. |
| Buffer | Supplied in PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3. |
| Species | Human |
| Storage | Store at -20℃. Avoid freeze / thaw cycles. |
| Synonyms | PDHA;PDHAD;PDHCE1A;PHE1A |
| Purification | Affinity purification |
| MolecularWeight | 43KDa |
| Description | |
| 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. |