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  • 產(chǎn)品名稱：HADHA 抗原（重組蛋白）

  • 產(chǎn)品型號：100ug
  • 產(chǎn)品廠商：通蔚生物
  • 產(chǎn)品價格：800
  • 產(chǎn)品庫存：35
  • 產(chǎn)品文檔：
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詳情介紹：



中文名稱： HADHA 抗原（重組蛋白）

英文名稱： HADHA Antigen (Recombinant Protein)

別     名：  hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit; GBP; ECHA; HADH; L

儲     存：  冷凍（-20℃）

相關類別： 抗原

概     述 

Fusion protein corresponding to a region derived from 514-763 amino acids of human HADHA

技術規(guī)格


Full name:

hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit

Synonyms:

GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA

Swissprot:

P40939

Gene Accession:

BC009235

Purity:

>85%, as determined by Coomassie blue stained SDS-PAGE

Expression system:

Escherichia coli

Tags:

His tag C-Terminus, GST tag N-Terminus

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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