Y39E4A.3 is orthologous to the human gene MAPLE SYRUP URINE DISEASE, TYPE IA (MSUD, TYPE IA. E1-alpha subunit of branched-chain keto acid dehydrogenase. OMIM:248600), which when mutated leads to maple syrup urine disease. Y39E4A.3 protein is predicted to be mitochondrial in Caenorhabditis elegans