General 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.[provided by RefSeq, Mar 2010]
Immunogen
A synthesized peptide derived from human PDHA1
Physical form
Buffer: PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
| biological source | rabbit |
| Quality Segment | 100 |
| conjugate | unconjugated |
| antibody product type | primary antibodies |
| clone | monoclonal |
| form | liquid |
| species reactivity | rat, human, mouse |
| concentration | 0.84 mg/mL |
| technique(s) | immunohistochemistry: 1:50-1:200,western blot: 1:500-1:2000 |
| UniProt accession no. | P08559 |
| shipped in | wet ice |
| storage temp. | −20°C |
| target post-translational modification | unmodified |
| Gene Information | human ... PDHA1(5160) |
