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 subunit beta, mitochondrial; BCKDE1B; branched chain alpha-ketoacid dehydrogenase E1-beta subunit; branched chain keto acid dehydrogenase E1, beta polypeptide; Branched-chain alpha-keto acid dehydrogenase E1 component beta chain; E1B dJ279A18.1 2-oxoisovalerate dehydrogenase beta subunit 2-oxoisovalerate dehydrogenase subunit beta mitochondrial BCKDE1B BCKDH E1-beta E1b-beta subunit of the branched-chain complex branched chain alpha-ketoacid dehydrogenase E1-beta subunit branched-chain alpha-ke; E1b-beta subunit of the branched-chain complex; testis secretory sperm-binding protein Li 240mP
Cat # 15754691
100 µL
Cat # 16842034
20 µL
Cat # 16506321
100 µL
Cat # 16165430
100 uL
Cat # 16832034
150 µL
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