BCKDK Antibody
CATALOG NUMBER: 62-652- Clonality: Polyclonal
- Tested Applications: WB
- Host Species: Rabbit
- Species Reactivity: Human, Mouse
- Conjugate: Unconjugated
- Size: 400 ul
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- HOST SPECIES: Rabbit
- SPECIES REACTIVITY: Human, Mouse
- HOMOLOGY: Predicted species reactivity based on immunogen sequence: Rat
- 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.
- CONJUGATE: Unconjugated
- TESTED APPLICATIONS: WB
- APPLICATION NOTE: For WB starting dilution is: 1:1000
- PREDICTED MOLECULAR WEIGHT: 46 kDa
Properties
- PURIFICATION: This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis
- CLONALITY: Polyclonal
- ISOTYPE: Rabbit Ig
- PHYSICAL STATE: Liquid
- BUFFER: Supplied in PBS with 0.09% (W/V) sodium azide.
- CONCENTRATION: batch dependent
- STORAGE CONDITIONS: 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.
Additional Info
- NCBI OFFICIAL SYMBOL: BCKDK
- ADDITIONAL NAMES: [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
- Protein Accession Number: O14874
- NCBI GENE ID NUMBER: 10295
- USER NOTE: Optimal dilutions for each application to be determined by the researcher.
Background
- 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.
Disclaimer
- FOR RESEARCH USE ONLY
For additional information, visit ProSci's Terms & Conditions Page. - Disclaimer: Optimal dilutions/concentrations should be determined by the end user. The information provided is a guideline for product use. This product is for research use only.
Shipping Info
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