Human/Mouse RGM-C Biotinylated Antibody

Catalog #: BAF3720 Datasheet / COA / SDS

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BAF3720

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Human/Mouse RGM-C Biotinylated Antibody Summary

Species Reactivity

Human, Mouse

Specificity

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.

Source

Polyclonal Goat IgG

Purification

Antigen Affinity-purified

Immunogen

Mouse myeloma cell line NS0-derived recombinant human RGM-C isoform a (R&D Systems, Catalog # 3720-RG)
Gln36-Asp400
Accession # Q6ZVN8

Formulation

Lyophilized from a 0.2 μm filtered solution in PBS with BSA as a carrier protein.

Label

Biotin

Purity

Antigen Affinity-purified

Applications

Recommended Concentration

Sample

Western Blot

0.1 µg/mL

Recombinant Human RGM-C (Catalog # 3720-RG)
Recombinant Mouse RGM-C (Catalog # 3634-RG)

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

Reconstitution Calculator

Preparation and Storage

Reconstitution

Reconstitute at 0.2 mg/mL in sterile PBS.

Shipping

The product is shipped at ambient temperature. Upon receipt, store it immediately at the temperature recommended below.

Stability & Storage

Use a manual defrost freezer and avoid repeated freeze-thaw cycles.

12 months from date of receipt, -20 to -70 °C as supplied.
1 month, 2 to 8 °C under sterile conditions after reconstitution.
6 months, -20 to -70 °C under sterile conditions after reconstitution.

Background: RGM-C/Hemojuvelin

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.

References

Papanikolaou, G. et al. (2004) Nat. Genet. 36:77.
Schmidtmer, J. and D. Engelkamp (2004) Gene Exp. Patterns 4:105.
Oldekamp, J. et al. (2004) Gene Exp. Patterns 4:283.
Niederkofler, V. et al. (2004) J. Neurosci. 24:808.
Niederkofler, V. et al. (2005) J. Clin. Invest. 115:2180.
Kuninger, D. et al. (2006) J. Cell Sci. 119:3273.
Zhang, A.S. et al. (2005) J. Biol. Chem. 280:33885.
Lin, L. et al. (2005) Blood 106:2884.
Huang, F.W. et al. (2005) J. Clin. Invest. 115:2187.
Krijt, J. et al. (2004) Blood 104:4308.
Babitt, J.L. et al. (2006) Nat. Genet. 38:531.

Long Name

Repulsive Guidance Molecule C

Entrez Gene IDs

148738 (Human); 69585 (Mouse); 310681 (Rat)

Alternate Names

DL-M; haemojuvelin; hemochromatosis type 2 (juvenile); Hemojuvelin; HFE2; HFE2AMGC23953; HJV; HJVHemochromatosis type 2 protein; JH; repulsive guidance molecule c; RGMC; RGM-C; RGMCRGM domain family member C