T05H10.6 encodes an ortholog of the human gene PYRUVATE DEHYDROGENASE (LIPOAMIDE) ALPHA 1 (PDHA1. OMIM-312170), which when mutated leads to Leigh syndrome (OMIM-256000). the T05H10.6 protein is predicted to be mitochondrial with 68% accuracy; The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components- pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3) (414 aa)