HADHA Polyclonal Antibody

Rs. 21,832.00
SKU E-AB-64551


Synonyms 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit,3 oxoacyl CoA thiolase,78 kDa gastrin binding protein,78 kDa gastrin-binding protein,ECHA,ECHA,GBP,HADH,HADHA,Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit,LCEH,LCHAD,Long chain 3-hydroxyacyl-CoA dehydrogenase,Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit,Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha subunit,Mitochondrial trifunctional enzyme alpha subunit,Mitochondrial trifunctional protein alpha subunit,MTPA,Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit,TP ALPHA,TP-alpha,Trifunctional enzyme subunit alpha mitochondrial precursor
Swissprot P40939
Source Rabbit
Reactivity Human,Mouse
Immunogen Recombinant protein of human HADHA
Application WB,IHC
Recommended dilution WB 1:500 - 1:2000 IHC 1:50 - 1:200
Concentration 1mg/ml
Clonality Polyclonal


Cellular localization  
Tissue specificity  
Isotype IgG
Purification Affinity purification
Conjugation Unconjugated
Storage instructions Store at -20℃. Avoid freeze / thaw cycles.
Storage buffer Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Background This gene encodes the alpha subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the alpha subunit catalyzing the 3-hydroxyacyl-CoA dehydrogenase and enoyl-CoA hydratase activities. Mutations in this gene result in trifunctional protein deficiency or LCHAD deficiency. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation.


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