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BCKDK Antibody (N-term)

Cat.#: T2003

Rabbit Polyclonal Antibody

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Description

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.

Recommended Applications

WB, ELISA

Applications and Recommended Dilution Factors

WB: 1:100~500
ELISA: 1:1,000

Species Reactivity

Human, Mouse

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Products Data

Western blot analysis of anti-BCKDK Antibody (N-term) in A2058 cell line lysates (35ug/lane).BCKDK arrow) was detected using the purified Pab.

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Specificity

This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 9~38 amino acids from the N-terminal region of human BCKDK.

Storage Condition and Buffer

Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C. Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.

Alternative Names

BCKDK antibody, BCKDKD antibody, BDK antibody, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial antibody, Branched-chain alpha-ketoacid dehydrogenase kinase antibody

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