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Branched-chain keto acid dehydrogenase is a multienzyme complex associated with the inner membrane of mitochondria, and functions in the catabolism of branched-chain amino acids. The complex consists of multiple copies of 3 components: branched-chain alpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2) and lipoamide dehydrogenase (E3). This gene encodes the E1 beta subunit, and mutations therein have been associated with maple syrup urine disease (MSUD), type 1B, a disease characterized by a maple syrup odor to the urine in addition to mental and physical retardation, and feeding problems.
2-oxoisovalerate dehydrogenase beta subunit; 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKD BCKDH E1-beta; BCKDE1B; BCKDH E1-beta; BCKDHB; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1 beta; branched chain keto acid dehydrogenase E1 subunit beta; branched chain keto acid dehydrogenase E1, beta polypeptide; branched chain ketoacid dehydrogenase E1, beta polypeptide; branched-chain alpha-keto acid dehydrogenase E1 component beta chain; dJ279A18.1; E1B; E1b-beta subunit of the branched-chain complex; testis secretory sperm-binding protein Li 240mP
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