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BCKDK Antibody (Center) 精选

Purified Rabbit Polyclonal Antibody (Pab)

     
  • 1 - BCKDK Antibody (Center) AP7112a
    Western blot analysis of BCKDK (arrow) using rabbit polyclonal BCKDK Antibody (Center) (Cat.#AP7112a). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2) (Origene Technologies).
  • 14 - BCKDK Antibody (Center) AP7112a
    Formalin-fixed and paraffin-embedded human cancer tissue reacted with the primary antibody, which was peroxidase-conjugated to the secondary antibody, followed by AEC staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated. BC = breast carcinoma; HC = hepatocarcinoma.
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Product Information
Application
  • Applications Legend:
  • E=ELISA
  • WB=Western Blotting
  • IHC=Immunohistochemistry
  • IHC-P=Immunohistochemistry (Paraffin)
  • IP=Immunoprecipitation
  • IF=Immunofluorescence
  • IC=Immunochemistry
  • ICC=Immunocytochemistry
  • FC=Flow Cytometry
  • DB=Dot Blot
WB, IHC-P, E
Primary Accession O14874
Other Accession Q00972, O55028
Reactivity Human
Predicted Mouse, Rat, Bovine, Canine, Rabbit
Host Rabbit
Clonality Polyclonal
Isotype Rabbit IgG
Antigen Region 120-151 aa
Additional Information
Other Names [3-methyl-2-oxobutanoate dehydrogenase [lipoamide]] kinase, mitochondrial, Branched-chain alpha-ketoacid dehydrogenase kinase, BCKD-kinase, BCKDHKIN, BCKDK
Target/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.
Dilution WB~~1:1000
IHC-P~~1:100~500
E~~Use at an assay dependent concentration.
Format Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.
StorageMaintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.
PrecautionsBCKDK Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.

For Research Use Only. Not For Use In Diagnostic Procedures.

Protein Information
Research Areas

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.

REFERENCES

Chang, C.F., et al., J. Biol. Chem. 277(18):15865-15873 (2002).
Popov, K.M., et al., J. Biol. Chem. 267(19):13127-13130 (1992).
Zneimer, S.M., et al., Genomics 10(3):740-747 (1991).

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