Recombinant Mouse RGM-C/Hemojuvelin Protein, CF

R&D Systems, part of Bio-Techne | Catalog # 3634-RG

R&D Systems, part of Bio-Techne
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3634-RG-050
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Key Product Details

Source

NS0

Accession #

Q7TQ32

Conjugate

Unconjugated

Applications

Binding Activity

View all RGM-C/Hemojuvelin Proteins and Enzymes »

Product Specifications

Source

Mouse myeloma cell line, NS0-derived mouse RGM-C/Hemojuvelin protein
Gln33-Asp393 (Ile379Val) (mature) & Pro166-Asp393 (Ile379Val) (C-terminus peptide), both with a C-terminal 6-His tag & Gln33-Asp165 (N-terminus peptide)

Purity

>90%, by SDS-PAGE under reducing conditions and visualized by silver stain.

Endotoxin Level

<0.01 EU per 1 μg of the protein by the LAL method.

N-terminal Sequence Analysis

Gln33 (mature & N-terminus peptide) & Pro166 (C-terminus peptide)

Predicted Molecular Mass

40 kDa (mature), 14 kDa (N-terminus peptide), 26 kDa (C-terminus peptide)

SDS-PAGE

57 kDa, 20 kDa and 38 kDa, reducing conditions

Activity

Measured by its binding ability in a functional ELISA.
Immobilized rhBMP-4 at 1 µg/mL (100 µL/well) can bind rmRGM-C with a linear range of 0.016-1 µg/mL.

Formulation, Preparation and Storage

3634-RG
Formulation Lyophilized from a 0.2 μm filtered solution in PBS.
Reconstitution
Reconstitute at 100 μg/mL in sterile PBS.

Reconstitution Buffer Available:
Size / Price
Qty
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.
  • 3 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). RGM-C is expressed in striated muscle and periportal hepatocytes (2-4). The protein undergoes partial cleavage intracellularly, resulting in a disulfide-linked dimer of the 14 kDa N-terminal and 33 kDa C-terminal portions (3, 5, 6). The N-terminal fragment contains an RGD motif, while the C-terminal fragment carries the GPI attachment site (3, 6). An alternatively spliced isoform lacks the N-terminal fragment. Full length RGM-C can also be released from the cell and circulates in the blood (5, 7). RGM-C is disrupted in type 2A juvenile hemochromatosis, a hereditary iron homeostasis disorder characterized by excessive iron accumulation (4). Loss of RGM-C function results in decreased expression of the iron regulatory hormone hepicidin and increased iron deposition in liver, pancreas, and heart (4, 8). Membrane associated RGM-C up-regulates hepicidin while soluble RGM-C down-regulates hepicidin expression (7). This appears to be an iron-responsive regulatory system, as high blood iron levels reduce the amount of soluble RGM-C produced (7). RGM-C, similar to RGM-A, associates with neogenin (6). Disease-related point mutations can prevent internal RGM-C cleavage or its ability to interact with neogenin (5, 6). Experimental inflammatory conditions result in decreased RGM-C expression and increased hepicidin expression, although the two effects occur independently (4, 9). RGM-C also functions as a BMP co-receptor and enhances BMP-2 and BMP-4 signaling (10). In this context, RGM-C enhances the BMP-2 up-regulation of hepatic hepicidin (10). Mature mouse RGM-C shares 89% and 97% amino acid (aa) sequence identity with human and rat RGM-C, respectively. It shares 51% and 44% aa sequence identity with mouse RGM-A and RGM-B, respectively.

References

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

Long Name

Repulsive Guidance Molecule C

Alternate Names

DL-M, Hemojuvelin, HFE2, HJV, JH, RGMC

Entrez Gene IDs

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

Gene Symbol

HJV

UniProt

Q7TQ32

Additional RGM-C/Hemojuvelin Products

Product Documents for Recombinant Mouse RGM-C/Hemojuvelin Protein, CF

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Product Specific Notices for Recombinant Mouse RGM-C/Hemojuvelin Protein, CF

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