SUMMARY PARAGRAPH for LAT1
LAT1 encodes the dihydrolipoamide acetyltransferase component (E2) of the yeast pyruvate dehydrogenase complex, which catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA (2, 1, 3, 4). Pyruvate dehydrogenase complexes are extremely large multienzyme structures, and the E2 subunit provides the central core, playing both an enzymatic and structural role (3). Multiple E2 (Lat1p) subunits act as a scaffold for the binding of pyruvate dehydrogenase (E1; Pda1p and Pdb1p), dihydrolipoamide dehydrogenase (E3; Lpd1p), and the Lpd1p-binding protein (Protein X; Pdx1p) (2, 3). The pyruvate dehydrogenase complex is located in the mitochondrial matrix (5). Cells lacking Lat1p are viable, but they lacked pyruvate dehydrogenase activity (2). Mutations in DLAT, the human dihydrolipoamide acetyltransferase gene, cause lactic acidosis (maple syrup urine disease, type II) (OMIM) (6).
Last updated: 2005-07-01