Automated description from the Alliance of Genome Resources
Exhibits alpha-ketoacid dehydrogenase activity. Predicted to be involved in branched-chain amino acid catabolic process and response to nutrient. Localizes to the mitochondrion. Human ortholog(s) of this gene implicated in maple syrup urine disease. Is expressed in several structures, including alimentary system; brain; genitourinary system; respiratory system; and sensory organ. Orthologous to human BCKDHB (branched chain keto acid dehydrogenase E1 subunit beta).