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.
仕様
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
Host：Rabbit
Reactivity：Human,Mouse
Applications：WB,IF
Concentration：1mg/ml
Immunogen：Recombinant protein of human HADHA
Purification Method：Affinity purification
Clonality：Polyclonal
Conjugation：Unconjugated
Buffer：Buffer： PBS with 0.02％ sodium azide, 50％ glycerol, pH7.3.
Dilution：WB1：500 - 1：2000 IF1：50 - 1：200
Gene_ID（human）：3030
Swissprot：P40939
Isotype：IgG
Research Areas：Cancer, Cardiovascular, Metabolism, Signal transduction, Tags ＆ Cell Markers
