Invitrogen™ Human BCKDK (aa 284-371) Control Fragment Recombinant Protein

Product Code. 30194209

Brand: Invitrogen™ RP106682

Description

Recombinant Protein

Highest antigen sequence indentity to the following orthologs: Mouse (97%), Rat (97%). This recombinant protein control fragment may be used for blocking experiments. 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.

The second major step in the catabolism of the branched-chain amino acids, isoleucine, leucine, and valine, is irreversibly catalyzed by the branched-chain alpha-keto acid dehydrogenase complex (BCKD), an inner-mitochondrial enzyme complex composed of 3 catalytic components: a branched-chain alpha-keto acid decarboxylase (E1), a dihydrolipoyl transacylase (E2), and a dihydrolipoamide dehydrogenase (E3). The complex also contains 2 enzymes that regulated the state of activity of the BCKD complex: a kinase (BCKDK), and a phosphorylase. The ubiquitously expressed kinase contains 1 histidine kinase domain. Maple syrup urine disease (MSUD) is a pathology secondary to an enzyme defect in the catabolic pathway of leucine, isoleucine, and valine. Accumulation of these amino acids and their corresponding keto acids results in encephalopathy and progressive neurodegeneration in infants not treated for MSUD.

Specifications

• Accession Number: O14874
• Concentration: ≥5.0 mg/mL
• For Use With (Application): Blocking Assay, Control
• Formulation: 1 M urea, PBS with no preservative; pH 7.4
• Gene ID (Entrez): 10295
• Name: Human BCKDK (aa 284-371) Control Fragment
• Quantity: 100 μL
• Regulatory Status: RUO
• Gene Alias: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; AI327402; BCKDHKIN; BCKDK; BCKDKD; BCKD-kinase; BDK; branched chain alpha-ketoacid dehydrogenase kinase; branched chain ketoacid dehydrogenase kinase; branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
• Common Name: BCKDK
• Gene Symbol: BCKDK
• Conjugate: Unconjugated
• Species: Human
• Recombinant: Recombinant
• Protein Tag: His-ABP-tag
• Sequence: TMESHLDTPYNVPDVVITIANNDVDLIIRISDRGGGIAHKDLDRVMDYHFTTAEASTQDPRISPLFGHLDMHSGAQSGPMHGFGFGLP
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Expression System: E. coli
• Form: Liquid
• Purity or Quality Grade: >80% by SDS-PAGE and Coomassie blue staining