Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
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產(chǎn)品名稱: Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
產(chǎn)品型號(hào): abx033187
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Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
Branched Chain Alpha-Ketoacid Dehydrogenase Kinase (BCKDK) Antibody
的詳細(xì)介紹
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.
| Target |
BCKDK |
| Reactivity |
Human |
| Host |
Rabbit |
| Clonality |
Polyclonal |
| Tested Applications |
WB, IHC |
| Recommended dilutions |
Optimal dilutions/concentrations should be determined by the end user. |
| Immunogen |
Human BCKDK. |
| Purification |
Purified Rabbit Polyclonal Antibody. |
| Isotype |
IgG |
| Conjugation |
Unconjugated |
| Specificity |
This BCKDK antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 120-151 amino acids from the Central region of human BCKDK. |
| Storage |
Aliquot and store at -20 °C. Avoid repeated freeze/thaw cycles. |
| Swiss Prot |
O14874
|
| Gene Symbol |
BCKDK |
| Buffer |
PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS. |
| UNSPSC Code |
12352203 |
| Availability |
Shipped within 5-10 working days. |
| Note |
This product is for research use only. |
