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Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
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Catalogue No: abx033169
Price:
US$326.25
(Size: 80 µl)
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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.
Research Articles on Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK)
| Target | Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) |
| Clonality | Polyclonal |
| Reactivity | Human, Mouse |
| Tested Applications | ELISA, WB |
| Host | Rabbit |
| Recommended dilutions | WB: 1/1000. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | KLH-conjugated synthetic peptide between 325-356 amino acids from the C-terminal region of human BCKDK. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS. |
| Storage | Aliquot and store at -20°C. Avoid repeated freeze/thaw cycles. |
| UniProt Primary AC | O14874 (UniProt, ExPASy) |
| UniProt Secondary AC | A8MY43, Q6FGL4, Q96G95, Q96IN5 |
| UniProt Entry Name | BCKD_HUMAN |
| Gene Symbol | BCKDK |
| KEGG | hsa:10295 |
| String | 9606.ENSP00000378405 |
| Molecular Weight | Calculated MW: 46.4 kDa |
| Buffer | PBS containing 0.09% sodium azide. |
| Specificity | Predicted to react with Rat BCKDK. |
| Availability | Shipped within 5-10 working days. |
| Note | This product is for research use only. |