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Antibody Product Overview
| ELK.No | ES11203 |
| Product name | RGMC rabbit pAb |
| Reactivity | Human;Rat;Mouse |
| Applications | WB;ELISA |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 148738 |
| Human Swiss-Prot | Q6ZVN8 |
| Source | Rabbit |
| Isotype | IgG |
| Target | RGMC |
| Fields | >>TGF-beta signaling pathway |
| Gene name | HFE2 HJV RGMC |
| Protein name | Hemojuvelin (Hemochromatosis type 2 protein) (RGM domain family member C) |
| Human gene link | |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | |
| Mouse gene link | |
| Mouse Swiss-Prot | Q7TQ32 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | Q8N7M5 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 270-350 |
| Specificity | RGMC Polyclonal Antibody detects endogenous levels of protein. |
| Formulation | Liquid in PBS containing 50% glycerol, and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 ELISA 1:5000-20000 |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | |
| Observed band (KD) | 46kD |
| Background | hemochromatosis type 2 (juvenile)(HFE2) Homo sapiens The product of this gene is involved in iron metabolism. It may be a component of the signaling pathway which activates hepcidin or it may act as a modulator of hepcidin expression. It could also represent the cellular receptor for hepcidin. Two uORFs in the 5' UTR negatively regulate the expression and activity of the encoded protein. Alternatively spliced transcript variants encoding different isoforms have been identified for this gene. Defects in this gene are the cause of hemochromatosis type 2A, also called juvenile hemochromatosis (JH). JH is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. [provided by RefSeq, Oct 2015], |
| Function | disease:Defects in HFE2 are the cause of hemochromatosis type 2A (HFE2A) [MIM:602390]; also called juvenile hemochromatosis (JH). JH is an early-onset autosomal recessive disorder due to severe iron overload resulting in hypogonadotrophic hypogonadism, hepatic fibrosis or cirrhosis and cardiomyopathy, occurring typically before age of 30. It is the consequence of intestinal iron hyperabsorption associated with macrophages that do not load iron. Deleterious mutations of HFE2 reduced HAMP (hepcidin) levels despite iron overload, which normally induces HAMP expression.,function:Involved in iron metabolism. May be a component of the signaling pathway which activates hepcidin (HAMP). May cooperate with hepcidin to restrict iron absorption in the gut. May act as a modulator of hepcidin expression, as deleterious mutations are associated with reduced hepcidin level. Could represent the cellular |
| Subcellular location | Cell membrane ; Lipid-anchor, GPI-anchor . Also released in the extracellular space. . |
| Expression | Adult and fetal liver, heart, and skeletal muscle. |
Additional Images
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Western blot analysis of lysates from K562 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES11203-50
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