Automated description from the Alliance of Genome Resources (Release 8.3.0)
Predicted to enable dihydrolipoamide branched chain acyltransferase activity and ubiquitin protein ligase binding activity. Predicted to be involved in branched-chain alpha-keto acid decarboxylation to branched-chain acyl-CoA. Located in mitochondrion. Is expressed in several structures, including alimentary system; central nervous system; integumental system; sensory organ; and urinary system. Used to study maple syrup urine disease. Human ortholog(s) of this gene implicated in maple syrup urine disease type II. Orthologous to human DBT (dihydrolipoamide branched chain transacylase E2).
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