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Invitrogen
Human BCKDK (aa 158-271) Control Fragment Recombinant Protein
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Product Details
RP-91406
Species
Expression System
Amino acid sequence
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Type
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Conjugate
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Product Specific Information
Highest antigen sequence indentity to the following orthologs: Mouse (99%), Rat (99%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-53697. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
References (0)
Bioinformatics
Protein Aliases: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial; BCKD-kinase; BCKDH kinase; BCKDHKIN; branched chain alpha-ketoacid dehydrogenase kinase; Branched-chain alpha-ketoacid dehydrogenase kinase; EC 2.7.11.4
Gene Aliases: BCKDK; BCKDKD; BDK
UniProt ID: (Human) O14874
Entrez Gene ID: (Human) 10295
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