| Species : |
Human |
| Source : |
E.coli |
| Tag : |
His |
| Description : |
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. |
| Form : |
Lyophilised. 0.5% Trehalose, 6M Urea, 100mM Sodium phosphate, 10mM Sodium chloride, pH 4.5. |
| AA Sequence : |
MRKMLAAVSRVLSGASQKPASRVLVASRNFANDATFEIKKCDLHRLEEGP PVT TVLTREDGLKYYRMMQTVRRMELKADQLYKQKIIRGFCHLCDGQE ACCVGLEA GINPTDHLITAYRAHGFTFTRGLSVREILAELTGRKGGCA KGKGGSMHMYAKN FYGGNGIVGAQVPLGAGIALACKYNGKDEVCLTLY GDGAANQGQIFEAYNMAA LWKLPCIFICENNRYGMGTSVERAAASTDY YKRGDFIPGLRVDGMDILCVREA TRFAAAYCRSGKGPILMELQTYRYH GHSMSDPGVSYRTREEIQEVRSKSDPIM LLKDRMVNSNLASVEELKEI DVEVRKEIEDAAQFATADPEPPLEELGYHIYSS DPPFEVRGANQWIKF KSVS |
| Applications : |
Western blot; Mass Spectrometry; SDS-PAGE |
| Stability : |
Upon delivery aliquot and store at -80 centigrade. Avoid freeze / thaw cycles. |
| Reconstitution : |
Reconstitute with 160 µl aqua dest. |