货号 | AF3720-SP |
别名 | DL-M; haemojuvelin; hemochromatosis type 2 (juvenile); Hemojuvelin; HFE2; HFE2AMGC23953; HJV; HJVHemochromatosis type 2 protein; JH; repulsive guidance molecule c; RGMCRGM domain family member C | 全称 | Repulsive Guidance Molecule C |
反应种属 | Human/Mouse |
应用 | Western Blot(0.1 µg/mL) Immunocytochemistry(5-15 µg/mL) |
目标/特异性 | Detects human and mouse RGM-C in direct ELISAs and Western blots. In direct ELISAs, approximately 10% cross‑reactivity with recombinant human (rh) RGM-B is observed and less than 5% cross-reactivity with rhRGM-A is observed. |
使用方法 | Western Blot: 0.1 µg/mL Immunocytochemistry: 5-15 µg/mL |
来源 | Reconstitute at 0.2 mg/mL in sterile PBS. |
产品组分 |
供应商 | R&D Systems |
Entrez Gene IDs | 148738 (Human); 69585 (Mouse); 310681 (Rat) |
应用文献 | |
R&D Systems personnel manually curate a database that contains references using R&D Systems products. The data collected includes not only links to publications in PubMed, but also provides information about sample types, species, and experimental conditions. Downregulation of hemojuvelin prevents inhibitory effects of bone morphogenetic proteins on iron metabolism in hepatocellular carcinoma. | |
纯化方式 | Antigen Affinity-purified |
免疫原 | Mouse myeloma cell line NS0-derived recombinant human RGM-C isoform a (R&D Systems, Catalog # 3720-RG) Gln36-Asp400 Accession # Q6ZVN8 |
生物活性 | Human, Mouse |
标记 | Unconjugated |
溶解方法 | Reconstitute at 0.2 mg/mL in sterile PBS. |
背景 | RGM-C, also known as hemojuvelin, is a member of the repulsive guidance molecule (RGM) family of GPI-linked neuronal and muscle membrane glycoproteins (1, 2). RGM-C is expressed in striated muscle and periportal hepatocytes (3-5). The protein undergoes partial cleavage intracellularly, resulting in a disulfide-linked dimer of the 14 kDa N-terminal and 33 kDa C-terminal portions (4, 6, 7). The N-terminal fragment contains an RGD motif, while the C-terminal fragment carries the GPI attachment site (4, 7). Two alternatively spliced isoforms lack either approximately half or the entire N-terminal fragment. Full length RGM-C can also be released from the cell and circulates in the blood (6, 8). RGM-C is disrupted in type 2A juvenile hemochromatosis, a hereditary iron homeostasis disorder characterized by excessive iron accumulation (5). In mouse, loss of RGM-C function results in decreased expression of the iron regulatory hormone hepicidin and increased iron deposition in liver, pancreas, and heart (5, 9). Membrane associated RGM-C upregulates hepicidin while soluble RGM-C downregulates hepicidin expression (8). This appears to be an iron-responsive regulatory system, as high blood iron levels reduce the amount of soluble RGM-C produced (8). RGM-C, similar to RGM-A, associates with neogenin (7). Disease-related point mutations can prevent internal RGM-C cleavage or its ability to interact with neogenin (6, 7). Experimental inflammatory conditions result in decreased RGM-C expression and increased hepicidin expression, although the two effects occur independently (5, 10). RGM-C also functions as a BMP coreceptor and enhances BMP-2 and BMP-4 signaling (11). In this context, RGM-C enhances the BMP-2 upregulation of hepatic hepicidin (11). Mature human RGM-C shares 89% amino acid (aa) sequence identity with mouse and rat RGM-C. It shares 49% and 44% aa sequence identity with human RGM-A and RGM-B, respectively. |
运输条件 | Blue Ice |
存放说明 | 4℃ |
参考文献 |
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RGM‑C/Hemojuvelin in HepG2 Human Cell Line. RGM‑C/Hemojuvelin was detected in immersion fixed HepG2 human hepatocellular carcinoma cell line using Goat Anti-Human/Mouse RGM‑C/Hemojuvelin Antigen Affinity-purified Polyclonal Antibody (Catalog # AF3720) at 10 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Goat IgG Secondary Antibody (red; Catalog # NL001) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. View our protocol for Fluorescent ICC Staining of Cells on Coverslips. |