Branched-chain keto acid dehydrogenase is a multienzymencomplex associated with the inner membrane of mitochondria, andnfunctions in the catabolism of branched-chain amino acids. Thencomplex consists of multiple copies of 3 components: branched-chainnalpha-keto acid decarboxylase (E1), lipoamide acyltransferase (E2)nand lipoamide dehydrogenase (E3). This gene encodes the E1 betansubunit, and mutations therein have been associated with maplensyrup urine disease (MSUD), type 1B, a disease characterized by anmaple syrup odor to the urine in addition to mental and physicalnretardation, and feeding problems.
应用类型
WB,FCM
免疫原
This BCKDHB antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 41-70 amino acids from the N-terminal region of human BCKDHB.
