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| Reactivity: | Human |
| Applications: | WB, IF/ICC, ELISA |
| Host Species: | Rabbit |
| Isotype: | IgG |
| Clonality: | Monoclonal antibody |
| Gene Name: | hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit alpha |
| Gene Symbol: | HADHA |
| Synonyms: | GBP; ECHA; HADH; LCEH; MTPA; LCHAD; TP-ALPHA; HA |
| Gene ID: | 3030 |
| UniProt ID: | P40939 |
| Clone ID: | 2V3B8 |
| Immunogen: | Recombinant fusion protein containing a sequence corresponding to amino acids 545-763 of human HADHA (NP_000173.2). |
| Dilution: | WB 1:1000-1:6000 |
| Purification Method: | Affinity purification |
| Concentration: | 1.12 mg/mL |
| Buffer: | PBS with 0.05% proclin300, 0.05% BSA, 50% glycerol, pH7.3. |
| Storage: | Store at -20°C. Avoid freeze / thaw cycles. |
| Documents: | Manual-HADHA monoclonal antibody |
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.
Images
 | Western blot analysis of various lysates, using [KO Validated] HADHA Rabbit mAb (A23892) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 20s. |
 | Western blot analysis of lysates from wild type(WT) and HADHA knockout (KO) 293T cells, using HADHA Rabbit mAb (A23892) at 1:1000 dilution. Secondary antibody: HRP-conjugated Goat anti-Rabbit IgG (H+L) (AS014) at 1:10000 dilution. Lysates/proteins: 25μg per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 20s. |
 | Confocal imaging of HeLa cells using HADHA Rabbit mAb (A23892,dilution 1:200)(Red). The cells were counterstained with α-Tubulin Mouse mAb (AC012,dilution 1:400) (Green). DAPI was used for nuclear staining (blue). Objective: 100x. |
 | Confocal imaging of PC-12 cells using HADHA Rabbit mAb (A23892,dilution 1:200)(Red). The cells were counterstained with α-Tubulin Mouse mAb (AC012,dilution 1:400) (Green). DAPI was used for nuclear staining (blue). Objective: 100x. |
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