Predicted to enable 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity and metal ion binding activity. Predicted to be involved in branched-chain amino acid catabolic process. Predicted to be located in mitochondrial matrix. Predicted to be part of oxoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha).
WB Description
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.