BCKDK Mouse anti-Human, Clone: OTI11C9, lyophilized, TrueMAB™

Mouse Monoclonal Antibody

Supplier:  OriGene CF500685

Catalog No. 50-165-9750

Description

Reconstitute with PBS (pH 7.3) and recommend to perform another round of desalting process using Product No. 7KMWCO

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 ubiquitiously 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

• Antigen: BCKDK
• Classification: Monoclonal
• Conjugate: Unconjugated
• Gene: BCKDK
• Gene Alias: BCKDKD, BDK
• Host Species: Mouse
• Purification Method: Affinity Chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 10295
• Content And Storage: -20° C, Avoid Freeze/Thaw Cycles
• Form: Lyophilized

• Applications: Immunocytochemistry, Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
• Clone: OTI11C9
• Formulation: PBS with 8% trehalose and no preservative; pH 7.3
• Gene Accession No.: O14874
• Gene Symbols: BCKDK
• Immunogen: Full length human recombit protein of human BCKDK produced in HEK293T cell.
• Quantity: 100 μg
• Primary or Secondary: Primary
• Target Species: Human
• Product Type: Antibody
• Isotype: IgG1