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BckdhB - Branched chain keto acid dehydrogenase E1 subunit beta

Predicted to enable catalytic activity. Predicted to contribute to 3-methyl-2-oxobutanoate dehydrogenase (2-methylpropanoyl-transferring) activity and branched-chain alpha-keto acid dehydrogenase activity. Predicted to be involved in branched-chain amino acid catabolic process and response to nutrient. Predicted to be located in mitochondrion. Predicted to be part of branched-chain alpha-ketoacid dehydrogenase complex and oxoglutarate dehydrogenase complex. Is expressed in adult head and adult heart. 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, Dec 2024]

Genes similar to BckdhB

NCBI Orthologs

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Species Gene Architecture aa