BCKDK antibody (Middle Region)
Quick Overview for BCKDK antibody (Middle Region) (ABIN359244)
Target
See all BCKDK AntibodiesReactivity
Host
Clonality
Conjugate
Application
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Binding Specificity
- Middle Region
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Specificity
- This antibody reacts to BCKDK.
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Purification
- Prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS
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Immunogen
- This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the central region of human BCKDK.
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Isotype
- Ig Fraction
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Application Notes
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ELISA: 1/1,000. Western blotting: 1/100 - 1/500. Immunohistochemistry: 1/50 - 1/100.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user. -
Restrictions
- For Research Use only
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Format
- Liquid
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Concentration
- 0.25 mg/mL
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Buffer
- PBS with 0.09 % (W/V) sodium azide
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Preservative
- Sodium azide
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Precaution of Use
- This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
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Handling Advice
- Avoid repeated freezing and thawing.
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Storage
- 4 °C/-20 °C
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Storage Comment
- Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at-20 °C for longer.
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- BCKDK (Branched Chain Ketoacid Dehydrogenase Kinase (BCKDK))
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Alternative Name
- BCKDK
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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.Synonyms: BCKD-kinase, BCKDHKIN, Branched-chain alpha-ketoacid dehydrogenase kinase, [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase mitochondrial
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Gene ID
- 10295, 9606
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UniProt
- O14874
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Pathways
- SARS-CoV-2 Protein Interactome
Target
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