Invitrogen™ BCKDK Polyclonal Antibody

Rabbit Polyclonal Antibody

Supplier:  Invitrogen™ PA5104476

Catalog No. PIPA5104476

Description

Antibody detects endogenous levels of total BCKD.

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: Affinity chromatography
• Regulatory Status: RUO
• Gene ID (Entrez): 10295, 12041, 29603
• Content And Storage: -20°C
• Form: Liquid

• Applications: Western Blot, Immunocytochemistry
• Concentration: 1 mg/mL
• Formulation: PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
• Gene Accession No.: O14874, O55028, Q00972
• Gene Symbols: BCKDK
• Immunogen: A synthesized peptide derived from human BCKDK(Accession O14874), corresponding to amino acid residues L21-T71.
• Quantity: 100 μL
• Primary or Secondary: Primary
• Target Species: Human, Mouse, Rat
• Product Type: Antibody
• Isotype: IgG

Safety and Handling

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