Name: HADHA 抗原（重組蛋白）
Brand: 通蔚生物

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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