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 mitochondrial alpha-ketoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Is an ortholog of human BCKDHA (branched chain keto acid dehydrogenase E1 subunit alpha).
Predicted to enable hydroxymethylglutaryl-CoA lyase activity. Predicted to be involved in ketone body biosynthetic process and leucine catabolic process. Human ortholog(s) of this gene implicated in amino acid metabolic disorder. Is an ortholog of human HMGCL (3-hydroxy-3-methylglutaryl-CoA lyase) and HMGCLL1 (3-hydroxy-3-methylglutaryl-CoA lyase like 1).