|Application ||WB, E|
|Other Accession||P26284, P29804, P35486, Q8HXW9, A7MB35, Q6P948|
|Predicted||Zebrafish, Bovine, Monkey, Mouse, Pig, Rat|
|Calculated MW||43296 Da|
|Antigen Region||226-255 aa|
|Other Names||Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial, PDHE1-A type I, PDHA1, PHE1A|
|Target/Specificity||This PDHA1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 226-255 amino acids from the Central region of human PDHA1.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||PDHA1 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle.|
|Cellular Location||Mitochondrion matrix.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Glushakova, L.G., et al. Mol. Genet. Metab. 98(3):289-299(2009)
Joao Silva, M., et al. Eur. J. Pediatr. 168(1):17-22(2009)
Boichard, A., et al. Mol. Genet. Metab. 93(3):323-330(2008)
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