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You are here:Home » Antibodies » Abs to Protein Kinase Substrates » Anti -BCKDK, CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase, Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase) (PE)

Anti -BCKDK, CT (T340) (3-methyl-2-oxobutanoate Dehydrogenase Lipoamide Kinase,
Mitochondrial, Branched-chain alpha-ketoacid Dehydrogenase Kinase, BCKDHKIN, BCKD-kinase)
(PE)

Pricing

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Specifications

Clone Host Grade Applications
Polyclonal Rabbit Affinity Purified E B
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.
Catalog #B0290-07A-PE
ApplicationsSuitable for use in ELISA and Western Blot. Other applications not tested.
Recommended DilutionELISA: 1:1,000
Western Blot: 1:100-1:500
Optimal dilutions to be determined by the researcher.
Storage and StabilityMay be stored at 4°C for short-term only. Aliquot to avoid repeated freezing and thawing. Do not freeze R-Phycoerythrin conjugates. Light sensitive. Aliquots are stable for at least 6 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Clone TypePolyclonal
IsotypeIgG
HostRabbit
SourceHuman
ConcentrationAs reported
FormSupplied as a liquid in PBS, pH 7.2. Contains ~0.09% sodium azide. Conjugated to R-Phycoerythrin (R-PE).
PurityPurified by Protein G affinity chromatography.
ImmunogenSynthetic peptide selected from the C-terminal region of human BCKDK (KLH).
SpecificityRecognizes human BCKDK. Species Crossreactivity: mouse.
Important NoteThis product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.


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