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Invitrogen
Human BCKDHB (aa 50-112) Control Fragment Recombinant Protein
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Product Details
RP-94643
Species
Expression System
Amino acid sequence
Tag
Class
Type
Purity
Conjugate
Form
Concentration
Purification
Storage buffer
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Storage conditions
Product Specific Information
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.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
References (0)
Bioinformatics
Protein Aliases: 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
Gene Aliases: BCKDE1B; BCKDH E1-beta; BCKDHB; E1B
UniProt ID: (Human) P21953
Entrez Gene ID: (Human) 594
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