| 克隆类型 : |
Rabbit Polyclonal IgG |
|
免 疫 原 : |
Peptide derived from human HADHA. |
|
纯化方式 : |
Immunogen affinity purified |
|
缓 冲 液 : |
PBS, pH 7.4(1%BSA and 0.1% Sodium azide) |
|
产品描述 : |
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 the3-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. |
| 适用物种 : |
Hu, Mo, Rat, Dog, Pig, Hor, |
| 应用说明 : |
WB: 1:200-1:1000 E: 1:500-1:1000 IHC: 1:200-1:1000 IF: 1:100-1:500 |
| 别 名 : |
HADHA; LCHAD; ECHA; GBP; HADH; LCEH; LCHAD; MTPA; TP-ALPHA; 3 ketoacyl Coenzyme A (CoA) thiolase alpha subunit; 3 oxoacyl CoA thiolase; 78 kDa gastrin binding protein; GBPv HADHv Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; LCHAD; Mitochondrial long chain 2 enoyl Coenzyme A (CoA) hydratase alpha subunit; Mitochondrial long chain L 3 hydroxyacyl Coenzyme A dehydrogenase alpha; Mitochondrial trifunctional enzyme alpha subunit; Mitochondrial trifunctional protein alpha subunit; MTPA; Thiolase/enoyl Coenzyme A hydratase (trifunctional protein) alpha subunit; TP ALPHA; Trifunctional enzyme subunit alpha mitochondrial precursor. |
| 保存条件 : |
Store at -20°C for one year. Avoid repeated freeze-thaw cycles. At 4°C for 2-3 weeks after reconstituted. |
| 标 记 物 : |
PLLABS公司可供应生物素、辣根过氧化物酶、荧光素标记抗体 |
|
产品说明 : |
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