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. PDHA1 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 PDHA1 are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for PDHA1蛋白別名為：PDHA1; PHE1A; Pyruvate dehydrogenase E1 component subunit alpha; somatic form, mitochondrial; PDHE1-A type I;基因ID為：5160；蛋白質ID:P08559
應用類型
WB，IHC-p，ELISA補充：最優的抗體稀釋比例需要基于客戶實驗進行優化.建議的起始稀釋比例如下: WB: 1:500-1:2000, IHC-p: 1:100-1:300, ELISA: 1:40000. Not yet tested in other applications.
免疫原
合成多肽：the C-terminal region of human PDHA1. at AA rangle: 290-370
