Application
Western blot analysis of BCKDK using rabbit polyclonal.293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2).| Product Name | BCKDK Antibody |
|---|---|
| Antibody Type | Primary Antibodies |
| Product 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. |
| Immunogen | 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. |
| Clonality | Polyclonal |
|---|---|
| Isotype | Ig |
| Host Species | Rabbit |
| Tested Applications | WB |
| For WB starting dilution is: 1:1000 | |
| Species Reactivity | Human |
| Concentration | 1mg/ml |
| Purification | Unpurified |
| Gene Symbol | BCKDK |
|---|---|
| Alternative Names | [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase mitochondrial Branched-chain alpha-ketoacid dehydrogenase kinase BCKD-kinase BCKDHKIN BCKDK |
| Molecular Weight(MW) | 46 kDa |
| Sequence Similarities | Predicted species reactivity based on immunogen sequence: Rat |
Application
Western blot analysis of BCKDK using rabbit polyclonal.293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2).| Application Notes | For WB starting dilution is: 1:1000 |
|---|
| Form | Liquid |
|---|---|
| Storage Instructions | Store at 4˚C for three months and -20˚C, stable for up to one year. As with all antibodies care should be taken to avoid repeated freeze thaw cycles. Antibodies should not be exposed to prolonged high temperatures. |
| Storage Buffer | Supplied in PBS with 0.09% (W/V) sodium azide. |
Data sheet for OM272322