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Antibody Product Overview
| ELK.No | ES15792 |
| Product name | HEM4 rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 7390 |
| Human Swiss-Prot | P10746 |
| Source | Rabbit |
| Isotype | IgG |
| Target | HEM4 |
| Fields | >>Porphyrin metabolism;>>Metabolic pathways;>>Biosynthesis of cofactors |
| Gene name | UROS |
| Protein name | HEM4 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 22276 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P51163 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human HEM4 AA range: 25-75 |
| Specificity | This antibody detects endogenous levels of HEM4 at Human/Mouse |
| 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) | 29kD |
| Observed band (KD) | |
| Background | The protein encoded by this gene catalyzes the fourth step of porphyrin biosynthesis in the heme biosynthetic pathway. Defects in this gene cause congenital erythropoietic porphyria (Gunther's disease). [provided by RefSeq, Jul 2008], |
| Function | catalytic activity:Hydroxymethylbilane = uroporphyrinogen III + H(2)O.,disease:Defects in UROS are the cause of congenital erythropoietic porphyria (CEP) [MIM:263700]; also known as Gunther disease. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. The manifestations of CEP are heterogeneous, ranging from nonimmune hydrops fetalis due to severe hemolytic anemia in utero to milder, later onset forms, which have only skin lesions due to cutaneous photosensitivity in adult life. The deficiency in UROS activity results in the non-enzymatic conversion of hydroxymethylbilane (HMB) into the uroporphyrinogen-I isomer.,disease:Severe congenital erythropoietic |
| Subcellular location | mitochondrion,cytosol, |
| Expression | Ubiquitous. |
Additional Images
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Western blot analysis of lysates from PC-12 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES15792-100
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