Product Specification
| Alternative Name: | Repulsive guidance molecule C, Hemojuvelin, Hemochromatosis type 2 protein, HFE2 |
| |
| Source: | Produced in HEK 293 cells. Human RGMC (aa 36-398) is fused at the N-terminus to a linker peptide (8 aa) and a FLAG®-tag. |
| |
| UniProt ID: | Q6ZVN8 |
| |
| Concentration: | 0.1mg/ml after reconstitution. |
| |
| Formulation: | Lyophilized. Contains PBS. |
| |
| Purity Detail: | Purified by single-step affinity chromatography. |
| |
| Endotoxin Content: | <0.1EU/µg purified protein (LAL test; Bio Whittaker). |
| |
| Reconstitution: | Reconstitute with 100µl sterile water. Further dilutions should be made with medium containing 5% fetal calf serum. |
| |
| Shipping: | Shipped on Blue Ice |
| |
| Long Term Storage: | -20°C |
| |
| Use/Stability: | Stable for at least 6 months after receipt when stored at -20°C. |
| |
| Handling: | Avoid freeze/thaw cycles. After reconstitution, prepare aliquots and store at -20°C. |
| |
| Scientific Background: | The RGMC (also called Hemojuvelin) is an GPI-anchored protein involved in the iron metabolism through regulation of hepcidin expression. Mutations in the gene encoding RGMC cause severe iron overload (juvenile hemochromatosis /hemochromatosis type 2A). The protein binds to neogenin and acts as co-receptor of the bone morphogenetic protein. The mature RGMc protein is proteolytically processed and glycosylated. |
| |
| Technical Info/Product Notes: | FLAG is a registered trademark of Sigma-Aldrich Co. |
| |
| Regulatory Status: | RUO - Research Use Only |
| |
General Literature References
Bone morphogenetic protein signaling by hemojuvelin regulates hepcidin expression: J.L. Babitt, et al.; Nat. Genet.
38, 531 (2006),
Abstract;
Hemojuvelin is essential for dietary iron sensing, and its mutation leads to severe iron overload: V. Niederkofler, et al.; J. Clin. Invest.
115, 2180 (2005),
Abstract;
RGM and its receptor neogenin regulate neuronal survival: E. Matsunaga, et al.; Nat. Cell Biol.
6, 749 (2004),
Abstract;
