| 货号 | BAF3720 |
| 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) |
| 目标/特异性 | Detects human and mouse RGM-C in Western blots. In this format, 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 |
| 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) |
| 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 | Biotin |
| 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 | -20℃ |
| 参考文献 |
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