Invitrogen™ BCKDK Recombinant Rabbit Monoclonal Antibody (HL2710)

Rabbit Recombinant Monoclonal Antibody

Brand:  Invitrogen™ MA556608

Product Code. 30275613

Description

BCKDK Recombinant Monoclonal Antibody for Western Blot, IHC (P)

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

• Antigen: BCKDK
• Classification: Recombinant Monoclonal
• Concentration: 1 mg/mL
• Formulation: PBS with no preservative
• Gene Accession No.: O14874, O55028, Q00972
• Gene Symbols: BCKDK
• Immunogen: Recombinant fragment of human BCKDK.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Clone: HL2710
• Conjugate: Unconjugated
• Gene: BCKDK
• 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
• Host Species: Rabbit
• Purification Method: Protein A
• Regulatory Status: RUO
• Gene ID (Entrez): 10295, 12041, 29603
• Content And Storage: -20°C, Avoid Freeze/Thaw Cycles
• Form: Liquid