Français
English
Antibody Product Overview
| ELK.No | ES15791 |
| Product name | HEM6 rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 1371 |
| Human Swiss-Prot | P36551 |
| Source | Rabbit |
| Isotype | IgG |
| Target | HEM6 |
| Fields | >>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors |
| Gene name | CPOX CPO CPX |
| Protein name | HEM6 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 12892 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P36552 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 304024 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q3B7D0 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human HEM6 AA range: 265-315 |
| Specificity | This antibody detects endogenous levels of HEM6 at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 50kD |
| Observed band (KD) | |
| Background | The protein encoded by this gene is the sixth enzyme of the heme biosynthetic pathway. The encoded enzyme is soluble and found in the intermembrane space of mitochondria. This enzyme catalyzes the stepwise oxidative decarboxylation of coproporphyrinogen III to protoporphyrinogen IX, a precursor of heme. Defects in this gene are a cause of hereditary coproporphyria (HCP).[provided by RefSeq, Oct 2009], |
| Function | catalytic activity:Coproporphyrinogen-III + O(2) + 2 H(+) = protoporphyrinogen-IX + 2 CO(2) + 2 H(2)O.,disease:Defects in CPOX are the cause of hereditary coproporphyria (HCP) [MIM:121300]. HCP is an acute hepatic porphyria and an autosomal dominant disease characterized by neuropsychiatric disturbances and skin photosensitivity. Biochemically, there is an overexcretion of coproporphyrin III in the urine and in the feces. HCP is clinically characterized by attacks of abdominal pain, neurological disturbances, and psychiatric symptoms. The symptoms are generally manifested with rapid onset, and can be precipitated by drugs, alcohol, caloric deprivation, infection, endocrine factors or stress. A severe variant form is harderoporphyria, which is characterized by earlier onset attacks, massive excretion of harderoporphyrin in the feces, and a marked decrease of coproporphyrinogen IX oxidase |
| Subcellular location | Mitochondrion intermembrane space. |
| Expression |
Additional Images
No image
Western blot analysis of lysates from PC-12 cells, primary antibody was diluted at 1:1000, 4°over night
No image
No image
No image
: AO-06-ES15791-100
: 10 Produits
Hurry! only 10 items left in stock.
Use collapsible tabs for more detailed information that will help customers make a purchasing decision.
Ex: Shipping and return policies, size guides, and other common questions.
- Paste the label on a flat surface on the package
- Make sure that both 1D and 2D barcodes are clearly visible
- Ensure that the label is smooth and isn’t creased or wrinkled
- Check for any tears, dents, holes or scratches
- Pack your product tightly, with the right size packaging
- Ensure both barcodes are on a flat surface of the package