+44 (0) 1223 755950
+1 832 327 7413
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
REQUEST MORE INFO
Catalogue No: abx033187
Price:
US$326.25
(Size: 80 µl)
Reviews: 
(0 Publications and Reviews)
(0 Publications and Reviews)
Click on the image to see the image legend
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 |
| Tested Applications | ELISA, WB, IHC |
| Host | Rabbit |
| Recommended dilutions | WB: 1/1000, IHC-P: 1/50 - 1/100. Not tested in IHC-F. Optimal dilutions/concentrations should be determined by the end user. |
| Conjugation | Unconjugated |
| Immunogen | KLH-conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK. |
| Isotype | IgG |
| Form | Liquid |
| Purification | Purified Rabbit Polyclonal Antibody. |
| 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 Mouse and Rat BCKDK. |
| Availability | Shipped within 5-10 working days. |
| Note | This product is for research use only. |