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.
应用类型
IF, IHC-P, WB
免疫原
Recombinant protein encompassing a sequence within the C-terminus region of human HADHA. The exact sequence is proprietary.
来源宿主
Rabbit
反应性
该HADHA 抗体的反应性请参考该产品的说明书
保存建议
Keep as concentrated solution. Aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.
其他
Aviva Systems Biology总部位于加利福尼亚州圣迭戈，在中国北京设有办公室，专注于为研究需求提供多克隆和单克隆抗体、ELISA试剂盒、蛋白质和定制服务。Aviva Systems Biology生产了24,000种经过验证的多克隆抗体，并提供近20,000种ELISA试剂盒，定制实验室服务包括蛋白表达和纯化、抗体开发，以及ELISA的开发、验证和生产。Aviva Systems Biology为与独特物种和靶标相关的研究提供独特工具，研究领域包括转录因子、癌症、心血管、细胞生物学、DNA损伤和修复、表观遗传学、信号转导、细胞分化、干细胞生物学等等。
