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Highest antigen sequence indentity to the following orthologs: Mouse (90%), Rat (90%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-56785. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。
蛋白别名: 2-oxoisovalerate dehydrogenase subunit beta, mitochondrial; BCKD BCKDH E1-beta; 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; dJ279A18.1; E1b-beta subunit of the branched-chain complex; testis secretory sperm-binding protein Li 240mP
基因别名: BCKDE1B; BCKDH E1-beta; BCKDHB; E1B
UniProt ID: (Human) P21953
Entrez Gene ID: (Human) 594