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Home Antibody All anti-BCKDK antibodies

Anti-BCKDK Antibody


Specifications Citations Customer Reviews Product Documents
SKU Description Amount Price Availability*  
TA302079 Rabbit Polyclonal Antibody against BCKDK (Center) 100ug $325 3-7 Days
LC401780 BCKDK HEK293T cell transient overexpression lysate (as WB positive control) 20ug $50 In Stock
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Also for BCKDK (NM_005881)
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OriGene Data

ImmunogenThis 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.
Clone Name IsotypeRabbit Ig
Species ReactivityHuman (Predicted: Mouse, Rat) Concentration0.25 mg/ml
Guaranteed Application *WB, IHC Suggested DilutionsWB: 1:1000, IHC: 1:50~100
Predicted MW Explanation 46360 Da
BufferPurified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide.
Purification This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Reference Data

Target NameHomo sapiens branched chain ketoacid dehydrogenase kinase (BCKDK), transcript variant 1
Alternative NameBCKDKD; BDK
Database LinkNP_005872
Entrez Gene 10295 Human
Entrez Gene 12041 Mouse
Entrez Gene 29603 Rat
FunctionThe 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.
Related PathwayProtein KinaseDruggable Genome

* Availability is in business days
* OriGene provides validated application data and protocol, with money back guarantee.

Western blot analysis of BCKDK (arrow) using rabbit polyclonal BCKDK Antibody (Center) (Cat.#TA302079). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected with the BCKDK gene (Lane 2) (Origene Technologies).
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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