SUMMARY PARAGRAPH for PDA1
PDA1 encodes the alpha subunit of pyruvate dehydrogenase (5, 1). Pyruvate dehydrogenase, consisting of alpha (Pda1p) and beta (Pdb1p) subunits, is the E1 component of the large multienzyme pyruvate dehydrogenase complex (3). This mitochondrial complex consists of an E2 (Lat1p) core that binds E1 (Pda1p and Pdb1p ), E3 (Lpd1p), and Protein X (Pdx1p) components (3). E1 (pyruvate dehydrogenase), E2 (dihydrolipoamide acetyltransferase), and E3 (dihydrolipoamide dehydrogenase) act in concert to convert pyruvate into acetyl-CoA (3). Cells lacking PDA1 are viable but lack pyruvate dehydrogenase activity, show slower growth on glucose, and exhibit increased formation of petites that lack mitochondrial DNA (rho0) (1, 6). The yeast PDA1 gene shows strong similarity to human PDHA1, mutations in which are responsible for most cases of pyruvate dehydrogenase deficiency (OMIM) (7).
Last updated: 2005-07-01