| 产品详情 |
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| Antigenic Specificity | BCKDK |
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| Clone | polyclonal |
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| Host Species | Rabbit |
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| Reactive Species | human, mouse |
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| Isotype | n/a |
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| Format | purified |
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| Size | 80 µl, 400 µl |
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| Concentration | n/a |
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| Applications | WB. RUO |
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| Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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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. This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 325-356 amino acids from the C-terminal region of human BCKDK. |
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| Immunogen | Human BCKDK. |
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| Other Names | BCKDK; [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase; mitochondrial; Branched-chain alpha-ketoacid dehydrogenase kinase; BCKD-kinase; BCKDHKIN |
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| Gene, Accession # | BCKDK, SwissProt: O14874 |
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| Catalog # | abx033169 |
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| Price | |
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| Order / More Info | BCKDK Antibody from ABBEXA |
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| Product Specific References | n/a |
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