Anti-BCKDK Rabbit Polyclonal Antibody

Supplier: Pierce Biotechnology
PA513903
CAPIPA5-13903EA 2561.45 CAD
CAPIPA5-13903
Anti-BCKDK Rabbit Polyclonal Antibody
This antibody is predicted to react with mouse and rat based on sequence homology. 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.

Immunohistochemistry: 1:50-1:100, Western Blot: 1:1000

Type: Primary
Antigen: BCKDK
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human
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