BCKDK Polyclonal Antibody, Invitrogen™

Rabbit Polyclonal Antibody

Brand:  Invitrogen PA531455

Product Code. 13475977

Description

Recommended positive controls: 293T, mouse brain, rat brain. Predicted reactivity: Mouse (99%), Rat (99%), Zebrafish (85%), Xenopus laevis (89%), Rhesus Monkey (98%), Bovine (98%). Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.

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: Polyclonal
• 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: Antigen affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 10295, 12041, 29603
• Content And Storage: Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
• Form: Liquid

• Applications: Immunohistochemistry (Paraffin), Western Blot
• Concentration: 0.8 mg/mL
• Formulation: 0.1M tris glycine with 20% glycerol and 0.01% thimerosal; pH 7
• Gene Accession No.: O14874, O55028, Q00972
• Gene Symbols: BCKDK
• Immunogen: Recombinant fragment corresponding to a region within amino acids 46 and 327 of Human BCKDK
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG