gene: bckd-1A [Browse genome (BioProject PRJNA13758)] [Search on AGR]
Caenorhabditis elegans 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).