Predicted to contribute to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity. Involved in response to cAMP; response to glucocorticoid; and response to nutrient. Predicted to be located in mitochondrion and nucleolus. Predicted to be part of mitochondrial alpha-ketoglutarate dehydrogenase complex. Human ortholog(s) of this gene implicated in maple syrup urine disease. Orthologous to human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta). [provided by Alliance of Genome Resources, Apr 2022]