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RGM-C/Hemojuvelin Antibody (751757) [Unconjugated]

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Recombinant Human RGM‑C/Hemojuvelin protein was serially diluted 2-fold and captured by Mouse Anti-Human RGM‑C/Hemojuvelin Monoclonal Antibody (Catalog # MAB3720) coated on a Clear Polystyrene Microplate ...read more

Product Details

Summary
Reactivity HuSpecies Glossary
Applications ELISA
Clone
751757
Clonality
Monoclonal
Host
Mouse
Conjugate
Unconjugated

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RGM-C/Hemojuvelin Antibody (751757) [Unconjugated] Summary

Immunogen
Mouse myeloma cell line, NS0-derived human RGM‑C/Hemojuvelin
Gln36-Asp400
Accession # Q6ZVN8
Specificity
Detects human RGM‑C/Hemojuvelin in direct ELISAs.
Source
N/A
Isotype
IgG2b
Clonality
Monoclonal
Host
Mouse
Purity Statement
Protein A or G purified from cell culture supernatant
Innovator's Reward
Test in a species/application not listed above to receive a full credit towards a future purchase.

Applications/Dilutions

Dilutions
  • ELISA

Packaging, Storage & Formulations

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.
Buffer
Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
Reconstitution Instructions
Reconstitute at 0.5 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for RGM-C/Hemojuvelin Antibody (751757) [Unconjugated]

  • 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

Background

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.

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

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

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