Thermo Fisher Scientific PDHA1 Monoclonal Antibody (9H9AF5)

Applications and Tested Dilutions

Product Specifications

Product Specific Information

For positive control, use isolated mitochondria from Human, Bovine, Rat, and Mouse hearts, HepG2 lysate; cultured normal Human embryonic lung fibroblasts (strain MRC5); HL60 cells.

Target Information

The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the conversion of pyruvate to acetyl-CoA and CO₂, linking glycolysis to the tricarboxylic acid (TCA) cycle.
The PDH complex includes three enzymatic components:

• Pyruvate dehydrogenase (E1)
• Dihydrolipoamide acetyltransferase (E2)
• Lipoamide dehydrogenase (E3)

The E1 enzyme is a heterotetramer consisting of two alpha and two beta subunits. The PDHA1 gene encodes the E1 alpha 1 subunit, which contains the active site essential for PDH complex function.
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 reported.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.